Malignant anemia was described in 1885 by Addison in his book on bronze disease of the adrenal glands under the name of idiopathic anemia and by Birmer in 1872 under the name of progressive malignant anemia.
For a long time, this disease was referred to as "primary" anemia. believing that its cause lies deep in the body. Already in 1870, on the basis of pronounced cases of complete atrophy of the digestive tract, the cause of anemia was considered to be the insufficiency of the digestive function. However, for many years the prevailing opinion was that "anemia occurs due to damage to the bone marrow by intestinal toxins or as a result of an oral ("oral") focal infection. Botkin established the importance for individual cases of malignant anemia of invasion with a wide tapeworm, and he explained the very mechanism of anemization as a reflex disturbance of the activity of the hematopoietic center in the brain. Achilles of the stomach, constantly accompanying malignant anemia, attracted the attention of scientists. Achilia develops, apparently, not so much as a result of inflammatory gastritis, but rather from neurodystrophic influence, especially with a lack of vitamins and protein in food. The development of anemia is often preceded by liver disease (cholecystitis, cholecystohepatitis), which can disrupt metabolism and worsen gastric trophism. Apparently, as a result of a persistent metabolic disorder and a violation of the nervous regulation of gastric secretion, under the influence of adverse influences, multiple cases of malignant anemia can also occur in one family. As a result of the same reasons, due to damage to the stomach in the family of a patient with malignant anemia, there may be cases of chlorosis, or in the patient himself, signs of this disease appear, as it were, to replace chlorosis. In the patient himself, it is possible to establish achilia ten years before the development of typical anemia. The method of gastroscopy was able to detect in patients areas of regeneration of the parenchyma of the stomach during remissions, especially under the influence of replacement therapy and intensive fortification, which to a certain extent explains the undulating course of the disease and shows the dependence of the course of malignant anemia on environmental factors. The glandular tissue of the stomach can also undergo further pathological restructuring and give rise to polypous growths and cancerous degeneration. The works of recent years have clarified the mechanism of development of anemia and lesions of the central nervous system, depending on the violation of gastrointestinal digestion and nutrition, based on the great therapeutic value of the hepatic diet in malignant anemia.
It should be considered proven that in a healthy person, in the process of gastric digestion, in addition to the breakdown of proteins by pepsin, the formation of a special anti-anemic substance occurs, which is absorbed in the intestines, is deposited, like glycogen, protein, in the liver and, as needed, is consumed in the bone marrow, ensuring normal maturation. erythrocytes. In patients with malignant anemia, this substance is not formed in the stomach, they do not have it in the liver either; therefore, hematopoiesis in the bone marrow does not go beyond megaloblasts and non-nuclear large erythrocytes (megalocytes), as in the fetus; with calf's liver or its extracts, patients receive this missing substance, which ensures the formation of normal normoblasts in the bone marrow, as well as non-nuclear erythrocytes.
Castle in 1928 made the following experiment, which proved the role of the stomach in hematopoiesis. When a patient with malignant anemia is fed meat digested in a thermostat with normal gastric juice, the blood composition quickly improves, while the gastric juice of a patient with malignant anemia, even with the addition of nepsin and hydrochloric acid, does not have such an effect. The active principle of meat, as well as those acting similarly to eggs, cereals, yeast, is probably close to the vitamins of the B complex. specific stomach enzyme) = heat-labile anti-anemic substance deposited in the liver.
A specific enzyme is produced in humans mainly by the fundic glands, and possibly only in trace amounts by other parts of the gastrointestinal tract. The chemical nature of the anti-anemic substance has not been elucidated, although in recent years a cobal-containing vitamin, vitamin B 12 , which is particularly active in this direction, has been isolated.
For the later stages of pernicious anemia, damage to the central nervous system is extremely characteristic in the form of degeneration of the posterior and lateral columns of the spinal cord, mainly in the cervical part; the development of this "funicular myelosis" is also associated with a digestive defect - underproduction in the stomach and intestines of a special substance, in the absence of which occurs, as in pellagra, demyelination and degeneration of the axial cylinders of the central neurons.
Severe anemia occurs with progressive atrophy of the stomach and intestines (malignant anemia in its own form and with sprue disease), after extensive resection of the stomach (“agastric” malignant anemia), with impaired absorption of the antianemic substance by the intestinal wall (gastrointestinal fistula, sprue) and increased its destruction inside the intestine (with stenosis of the small intestine, with infection with a wide tapeworm). Malignant anemia can be detected in pregnant women due to increased consumption of hematopoietic substance for hematopoiesis of the fetus, and finally, due to a lack of an external factor (appropriate vitamins) with prolonged malnutrition.
The closest mechanism for the development of anemia is presented in the following form. Defective erythrocytes released by the bone marrow, as well as pathological erythrocytes in hemolytic jaundice, are easily destroyed in the spleen and other places of accumulation of reticuloendothelial tissue. The number of erythrocytes progressively falls, despite hyperplasia of the active bone marrow.
The consequence of increased breakdown of erythrocytes is yellowness of patients, increased levels of indirect bilirubin in the blood, urobilin in feces and urine, however, in a much smaller amount than with hemolytic jaundice, due to a deeper violation of pigment metabolism with the formation of porphyrin and hematin. In the blood serum, the content of iron is increased, which is deposited in Kupffer cells and in the parenchyma on the periphery of the liver lobules, in the lungs, spleen, kidneys - homosiderosis of the organs, giving them a rusty tint. Anatomically characteristic is a crimson-red brain in the diaphysis of tubular bones, for example, the thigh, rich in megaloblasts, with scanty foci of formation of granular leukocytes and megakaryocytes. The same metaplasia can be in a mildly enlarged spleen. At autopsy, fatty degeneration of the liver, degeneration of the kidneys, hemorrhages in the serous membranes, retina, and brain are found.
During remission, megaloblasts are replaced by erythroblasts, normoblasts and ordinary erythrocytes, pathological hemolysis stops, the need for colossal hematopoiesis decreases, the bone marrow of the thigh acquires a normal fatty appearance, and the sternum punctate does not contain megaloblasts after several days of liver treatment.

clinical picture. Patients complain of gradually increasing general weakness, shortness of breath, palpitations, dizziness, pain in the heart, often swelling of the legs or general swelling, fever. At first glance, the doctor's attention is attracted by the general appearance of the patient: in front of him is an elderly man who looks older than his years, or a woman 30-40-50 years old, often graying early with bloodless mucous membranes and skin of a pale lemon-yellow hue; patients are not very emaciated, often even full. On the face, trunk, hands, pigmented spots (chloasma) and areas of depigmentation (vitiligo), wen. Patients often come with a diagnosis of heart disease, cardiosclerosis, angina pectoris or stomach cancer. With a detailed questioning, it is possible to find out that the patient has had burning pains in the tongue from spicy food for the last few months, periodically diarrhea, and when examining gastric juice, achylia has long been established; that before, for example, towards the end of last winter, the pallor of the patient attracted the attention of others, and a blood test established anemia; lately, the patient's fingertips have become numb and there is a sort of crawling and tingling in the hands and feet (paresthesia). In history, often, especially in women, attacks of hepatic colic, cholecystohepatitis.
Edema can reach the degree of anasarca with ascites, hydrothorax, edematous pads on the back of the hands, on the lower back and sacrum, sometimes swelling is limited to the shins, puffiness of the face. With very severe anemia, a few petechiae are found on the skin and minor hemorrhages in the mucous membranes. With pressure, soreness of the sternum (sternalgia), ribs.
The cardiovascular system presents the usual changes for severe anemia: increased cardiac activity with increased pulsation of the carotid and other arteries with an increase in pulse pressure; sharp systolic murmur at all orifices of the heart from eddy movements of blood with reduced, often two times against the norm, viscosity, with a sharp acceleration of blood flow. Those conditions cause a murmur audible on the bulb of the jugular vein, better on the right. This continuous whirring noise increases with inhalation, when the venous blood rushes with even greater force into the chest. The mass of blood is only slightly reduced: the loss of erythrocyte mass is replenished, as in general with anemia, by an increase in the liquid part of the blood. The acceleration of blood flow and other adaptive mechanisms of blood circulation provide a more or less normal respiratory function of the blood; this must be seen as an explanation for the amazing fact that patients with severe malignant anemia are still often capable of hard physical work. Edemas are, as a rule, “protein-free” in nature, that is, they are associated with a low content of protein in the blood serum. With prolonged anemia on the basis of anoxemia, fatty degeneration of organs develops, including the “tiger heart”. In this case, there may be signs of true circulatory insufficiency with an expansion of the cavities of the heart, an increase in venous pressure, a slowdown in blood flow, etc. In severe anemia, attacks of angina pectoris of a functional apoxemic nature may begin, which stop later along with an improvement in the composition of the blood. Of course, in elderly patients one has to reckon with the possibility of angina pectoris on the basis of coronary sclerosis.
On the part of the digestive organs, the tongue is characteristic - clean, bright red, smooth, devoid of papillae, atrophic, sometimes covered with aphthous vesicles or superficial sores (glossitis). Burning pain in the tongue is sometimes the main complaint of patients. The same burning pains can be in the esophagus due to a similar esophagitis. Dyspeptic complaints from the stomach are uncharacteristic, although due to the atrophic process, the secretion of hydrochloric acid cannot be caused by a subcutaneous injection of 0.5 mg of histamine, i.e., there is a histamine-resistant achilia (The absence of a hematopoietic enzyme in the patient's gastric juice can be proved by biological tests: 1) repeated feeding another patient with undoubted pernicious anemia in the stage of exacerbation of meat, each time subjected to digestion in a thermostat with the gastric juice of the patient under study, does not cause remission; 2) subcutaneous administration of neutralized gastric juice of the patient to rats, unlike normal juice, does not cause an increase in the number of reticulocytes (the so-called rat-reticulocyte test). With extreme degrees of anemization, there may be persistent vomiting, making it impossible to ingest the liver. Periodically advancing diarrhea is in the nature of enteritis, accompanied by swelling, rumbling, pain near the navel.
The liver during exacerbations is usually enlarged; often sensitivity in the gallbladder and other signs of cholecysto-hepatitis.
A slight enlargement of the spleen is noted in most untreated cases.
From the side of the central nervous system, signs of damage to the predominantly posterior columns of the spinal cord are found - a decrease and absence of reflexes, ataxia, a significant violation of deep sensitivity while maintaining superficial ("pseudotabes"); the lateral columns are also affected to a lesser extent, and spastic phenomena predominate and often, disorders of superficial sensitivity are detected early. More often, spinal cord lesions are mixed, with a predominance of the posterior columnar type. Flaccid or spastic paralysis of the lower extremities develops, later the sphincters of the bladder and rectum are disturbed. Less commonly observed are degenerative processes in the brain with a varied picture of general asthenia, psychosis, and peripheral neuritis. Patients often complain of memory impairment, irritability.
When the number of erythrocytes falls to 2,000,000 or below, there is almost always a significant fever of one or another, usually of the wrong type, simulating malaria, typhoid fever, sepsis, and other infectious diseases. An increase in temperature can reach 39-40 ° and is apparently associated with a sharp breakdown of the blood or rejuvenation of the bone marrow; this aseptic "anemic" fever quickly stops with an improvement in the composition of the blood.
The most characteristic blood changes are as follows. There is a high color index, greater than one, which depends on the presence of large macrocytic erythrocytes that are excessively stained with hemoglobin (hyperchromia), which creates a misleading impression of a good blood composition on a cursory examination of the smear (as opposed to iron deficiency anemia, in which erythrocytes remain completely unstained in the center ). So, at 40% hemoglobin, 1,500,000 red blood cells (30% of the norm) are often determined, i.e., the color indicator is 1.3. The color indicator remains high for a long time and with a significant improvement in red blood, without falling, as a rule, and in remissions below 0.7-0.8, which is in sharp contrast to the low color indicator of patients with chlorosis. On the part of white blood, leukopenia is characteristic with polysegmented neutrophils - five-, seven-lobed, etc. - the so-called shift to the right in the neutrophilic series.

Careful examination of a stained smear reveals a variety of other cellular forms: pathognomonic megaloblasts with a characteristic sieve-like structure of the nucleus (“like raindrops in sand”) and often basophilic protoplasm; megalocytes - non-nuclear erythrocytes - giants over 12 r. diameter; poikilocytes (erythrocytes of the most bizarre shape - in the form of a tennis racket, tape); fragments of erythrocytes, polychromatophiles, erythrocytes with basophilic puncture, with Cabot rings, Jolly bodies, neutrophilic myelocytes, etc.

An increase in the number of reticulocytes, eosinophils, platelets indicates the restoration of normal bone marrow hematopoiesis and is a good indicator of the onset of remission. Liver therapy naturally causes a steep rise in the number of reticulocytes on the 7-10th day with a slower subsequent decline. Following the peak of reticulocytes from the 10-14th day of treatment, a constant long-term rise in the number of erythrocytes and hemoglobin occurs, which continues after the fall of reticulocytosis. The number of eosinophils in the treatment of raw liver remains extremely high for a long time, reaching 50-60% of all leukocytes; liver extracts give only moderate eosinophilia.

Turning, complications and outcomes. The onset of the disease is gradual. Obviously, at least half a year or a year passes before the blood drops to those low figures at which the patient usually goes to the doctor. A significant deterioration in the condition is often preceded by attacks of glossitis with diarrhea, as in other diseases of insufficiency, or an accidental acute infection.
Prior to the introduction of hepatic therapy, the disease fully justified its name as pernicious (“fatal”) and progressive. True, even without treatment, the disease gave one or two, rarely three characteristic remissions lasting from 2-3 months to 1 year, yet leading to death usually after 1-3 years, as an exception, after 7-10 years. Treatment with the liver significantly changed the course of the disease, making it possible to immediately induce remission at the first attack and maintain this state of practical health and full working capacity, apparently for an indefinitely long time, at least up to 10-15 years. However, the disease remains serious due to the often late recognition of many complications (pyelitis, cholecystitis, protein-free edema) and especially due to the difficulty of long-term liver replacement treatment; in the end, however, the disease leads to severe damage to the spinal cord with paralysis, urinary infection (urosepsis), pneumonia, and death.

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A severe attack, not recognized and not treated in a timely manner, can lead to a kind of coma - coma perniciosum, when in patients with a hemoglobin amount of about 10% (hard to determine exactly), general edema, jaundice, hypothermia, circulatory disorders, sharp lethargy, drowsiness occurs, even complete loss of consciousness, and it is possible to get the patient out of this dangerous state only by urgent measures: drop blood transfusion, parenteral administration of active hepatic drugs in a large dose, cardiovascular agents, oxygen therapy.
A completely different picture is presented by patients in a state of remission, when, in the complete absence of complaints, neither the appearance of the patient and the study of individual organs (normal color of the skin and mucous membranes, an undischarged spleen and liver), nor a blood test (normal number of erythrocytes and hemoglobin; the most persistent morphological changes are macrocytosis with hyperchromia and leuko-neutropenia, but they, apparently, can disappear; even punctate of the sternum reveals normoblastic bone marrow). But this well-being is under constant threat from the remaining disturbed activity of the nervous system and digestive tract: attacks of diarrhea, exacerbation of glossitis, the development of polyposis and stomach cancer, and most importantly, nervous symptoms can progress. Even with a normal blood composition, the patient can become a severe invalid, bedridden due to paraplegia, etc. Ignoring liver therapy can also lead to a relapse of anemia.

Pernicious-like macrocytic a blood picture without other classic signs of malignant anemia can cause diseases such as cirrhosis of the liver, malaria, acute leukemia, etc. Chronic leukemia, especially lymphatic, is sometimes combined with pernicious anemia - actually "leukanemia". With family hemolytic jaundice, anemia is hyperchromic, but at the same time microcytic in nature; this disease is distinguished by a peculiar, well-defined clinical picture.

A plastic anemia (aleukia), as well as acute leukemia are often misdiagnosed as "acute pernicious anemia". Both diseases have a very similar clinical picture, which differs, however, significantly from pernicious anemia; there are also significant hematological differences.

cancer anemia, for example, in gastric cancer, it is usually easy to differentiate from malignant anemia by a low color index, neutrophilia with a normal or elevated white blood cell count, the presence of persistent gastric complaints, and most importantly, by a defect in gastric filling on an x-ray. X-ray examination, on the other hand, makes it possible to recognize polyposis of the stomach, often also giving severe anemia.
It should, however, be noted that in case of malignant anemia, swelling of the gastric mucosa can give an x-ray picture similar to a filling defect, and most importantly, with a long course, malignant anemia can lead to true gastric neoplasm. This combination can be recognized, in addition to a stable x-ray picture, by a continuous worsening of the patient's condition with a sharp cachexia, unusual for malignant anemia, a positive blood symptom - blood in the stool, in vomit, leukocytosis (and thrombocytosis). In isolated early cases, such patients underwent a radical operation to remove the tumor, and they recovered with liver replacement therapy.
In addition to cancer, other severe hypochromic anemias are often incorrectly mistaken for malignant anemia, such as: anemia with prolonged hemorrhoidal bleeding, with chronic azotemic nephritis (anemia of the brights). According to the first impression (pale face, swelling of the eyelids and other signs of sclerotic cachexia), an elderly patient with atherosclerosis, as well as patients with edematous disease or lipoid-nephrotic syndrome, can be mistaken for a patient with pernicious anemia, but with these diseases, red blood is almost not affected .
Due to the presence of fever, along with swelling of the spleen, leukopenia, anemia, pernicious anemia is sometimes misdiagnosed as malaria (bright large basophilic puncture and Kebot rings in erythrocytes are often mistaken for the stage of development of malarial plasmodium), typhoid fever (diarrhea contributes to the misdiagnosis, leukopenia, etc.), sepsis-endocarditis lenta (heart murmurs, arterial pulsation, enlarged spleen, etc.).
Finally, often a significant damage to the spinal cord in malignant anemia leads to an erroneous diagnosis of the actual nervous suffering, especially the dorsal tabes (tabes dorsalis), multiple sclerosis, tumors of the spinal cord, polyneuritis; this mistake is even easier to make if after treatment with the liver only an erased anemic syndrome remains. There are known forms of funicular myelosis due to insufficiency of gastrointestinal digestion without anemia at all. In contrast to the dorsal tabes in malignant anemia, the cerebrospinal fluid remains normal, pupillary reactions, as a rule, persist, motor muscle strength is sharply disturbed, and shooting pains are unusual. The difference from multiple sclerosis is the older age of patients, the absence of eye symptoms and damage to the optic nerve, the absence of deep reflexes, the violation of sphincters only in the later stages of the disease.

Prevention. It should be remembered that every patient with persistent achilia, as well as after extensive resection of the stomach, may develop severe anemia, therefore, in such persons, it is necessary to systematically monitor the general condition and blood composition (hyperchromia, macrocytosis, etc.), provide them with a complete diet, and if necessary, apply early treatment of anemic syndrome and other measures. Thus, it will be possible to reduce the number of patients with malignant anemia who seek medical attention for the first time with severe anemia (often with a red blood cell count of about 2,000,000), requiring hospitalization and leading to long-term disability. The general correct hygienic regimen, the protection of the nervous system from difficult experiences are also of undoubted importance in the prevention of the disease.

Treatment. The treatment of malignant anemia is aimed primarily at regulating the general regimen, improving the neurotrophic regulation of the digestive organs and other systems, eliminating various foci of irritation (infectious diseases, inflammation of the gallbladder, etc.), providing good nutrition, especially substances that are most closely related to trophic - the gastrointestinal tract, hematopoietic organs and the nervous system (separate fractions of vitamin Bb, autoclaved yeast, etc.). During an exacerbation of the disease, bed rest is necessary. The food is varied, sufficient, always with a normal amount of high-grade proteins - meat, eggs, milk; the same principle of nutrition is observed in extreme degrees of anemia and the presence of diarrhea - meat soufflé, beaten egg whites, freshly grated cottage cheese, yogurt, etc.
The liver is both a food and the best remedy; the usual dose of it is 200 g per day. Fresh liver (veal, bovine) is washed in warm water, cleaned of connective tissue fibers, passed through a meat grinder and given raw or lightly scalded on the outside with boiling water on a sieve, with any seasonings, depending on the taste of the patient - with salt, onion, pepper, garlic, as well as in the form of sandwiches, sweet dishes, etc. At the first time, when the treatment of malignant anemia with the liver was proposed (1926), it was considered necessary to prescribe a special diet, poor in fats and rich in fruits. Soon, however, it became clear that treatment with the liver is valid with any diet, although it is advisable to provide the patient with a complete protein diet. The addition of hydrochloric acid is desirable, as with any achilia. Raw liver does not pose a danger in relation to helminthic invasion; often developing very high eosinophilia is a consequence of the intake of raw liver as such. Diarrhea is not a contraindication to raw liver treatment, on the contrary, with such treatment, it usually stops soon.
The various oral liver concentrates—liquid, dried powder—are practically of lesser value; they do not always satisfy the taste of patients, and most importantly, they are not always sufficiently active; one of the best is liver extract, given at a dose of about 2 tablespoons per day. The therapeutic effect is also possessed by "gastrocrine", a dried pig stomach, containing, in addition to the internal factor formed in pigs mainly by the pyloric glands, also the external one - from the muscular layers of the stomach.
Fresh veal liver is a reliable carrier of an antianemic substance; in addition, it is rich in vitamins, iron, copper. If the patient refuses to take the liver orally, as well as in a severe, life-threatening condition, and, of course, with a sharp violation of intestinal absorption, parenteral administration of liver preparations should be preferred.
Active injectable drugs, for example, Soviet campolon, gepalon, hepatia, are administered intramuscularly, usually 1-2 ml, and in the most severe cases up to 5-8 ml per day (the so-called "campolon shock" treatment) until the onset of a clear remission. In the future, a rapid increase in red blood to normal numbers is ensured, which requires at least 2-4 intramuscular injections of 1-2 ml of the active drug per week or daily intake of raw liver. In cases of moderate severity (when the number of erythrocytes is about 1,500,000 and hemoglobin is about 40%), the patient's well-being improves significantly by the end of the first week of treatment. On the 7-10th day, a peak of reticulocytosis is noted, followed by an increase in the number of erythrocytes and hemoglobin, which occurs the more vigorously, the lower the initial numbers were. Having achieved a stable remission in the treatment of raw liver, they often take a break for several weeks, and in the treatment of injections they are content with 1-2 injections per week or even more rare.
In cases of apparent invalidity of hepatic therapy, the misdiagnosis of malignant anemia or insufficient liver dosage is more common. So, with infectious complications - pyelitis, pneumonia, as well as in some less treatable patients, a double dose of 400 g of raw liver per day may be required. In these cases, as well as with extreme anemia, an additional drop blood transfusion is indicated repeatedly, 100-150 ml or more, or, better, erythrocyte mass (suspension) of 100-200 ml.
Recently, it has been recommended to use vitamin B12 treatment, which is already active in very small doses and, moreover, has a good effect on blood restoration and, if given in a timely manner, apparently prevents damage to the nervous system. (Another anti-anemic vitamin, folic acid, does not prevent neurological manifestations of the disease and is therefore now considered less indicated in malignant anemia.)
To prevent and improve nerve damage, a valuable adjunct to liver treatment, especially in the treatment of liver injections, is the systematic administration of vitamin B 1 (thiamine under the skin or inside, yeast autoclaved in a thermostat together with gastric juice); with spastic paralysis, motor-mechanotherapy is carried out, training in movements, walking, as in tabes, etc.
Arsenic currently does not find supporters in the treatment of malignant anemia; iron is indicated especially in the presence of iron deficiency anemia.
In cases of symptomatic and malignant anemia - with a wide tapeworm, pregnancy, agastric anomia, etc. - also, first of all, energetic treatment is carried out with raw liver or injections of appropriate drugs (injections are required for sprue, gastrocolic fistula, when absorption from the intestine is obviously extremely reduced), blood transfusion. In this way, it is possible to bring pregnant women to normal delivery, with an invasion with a wide tapeworm, significantly improve the composition of the blood and the entire clinical picture, followed by fern treatment, which is contraindicated in patients with a serious condition due to its general toxic effect and liver damage caused by it; with gastrocolic fistula, with stenosis of the small intestine, early surgical intervention is indicated, until severe cachexia occurs.

Blood picture: hyperchromic type anemia, macrocytes, megalocytes, with Jolly bodies, Cabot rings, leukopenia, (during an exacerbation).

Treatment carried out -100-200 mcg intramuscularly daily or every other day before the onset. In the event of an anemic coma - urgent hospitalization, better than erythrocyte mass (150-200 ml). Maintenance therapy with vitamin B12 is necessary to prevent relapse. A systematic monitoring of the composition of the blood in people with persistent achilia, as well as those who have undergone, is shown. Patients suffering from pernicious anemia should be under dispensary observation (possible occurrence of stomach cancer).

1. malignant anemia(synonym: pernicious anemia, Addison-Birmer disease). Etiology and pathogenesis. Currently, pernicious-anemic syndrome is considered as a manifestation of B12-avitaminosis, and Addison-Birmer disease is considered as endogenous B12-avitaminosis due to atrophy of the fundic glands that produce gastromucoprotein, resulting in impaired absorption of vitamin B1a, which is necessary for normal, normoblastic, hematopoiesis, and pathological, megaloblastic, hematopoiesis develops, leading to anemia of the "pernicious" type.

Clinical picture (symptoms and signs). Persons aged 40 45 are ill more senior. Characterized by violations of the cardiovascular, nervous, digestive and hematopoietic systems. Complaints of patients are diverse: general weakness, shortness of breath, palpitations, pain in the region of the heart, swelling of the legs, dizziness, crawling in the hands and feet, gait disorder, burning pain in the tongue and esophagus, periodic diarrhea. The appearance of the patient is characterized by pale skin with a lemon-yellow tint. The sclera are subicteric. The patients are not exhausted. The face is puffy, swelling in the ankles and feet. Edema can reach high degrees and be accompanied by ascites, hydrothorax. On the part of the cardiovascular system - the appearance of systolic murmur at all openings of the heart and the noise of the "top" on the bulb of the jugular vein, which is associated with a decrease in blood viscosity and an acceleration of blood flow; possible anoxemic angina pectoris. With prolonged anemia, fatty degeneration of organs develops, including the heart (“tiger heart”), as a result of persistent anoxemia. On the part of the digestive organs - the so-called hunter's (gunter's) glossitis, the tongue is clean, bright red, devoid of papillae. Analysis of gastric juice, as a rule, reveals histamine-resistant achilia. Periodic diarrhea is a consequence of enteritis. The liver is enlarged, soft; in some cases - a slight increase in the spleen. With a significant drop in the number of red blood cells (below 2,000,000), a fever of the wrong type is observed. Changes in the nervous system are associated with degeneration and sclerosis of the posterior and lateral columns of the spinal cord (funicular myelosis). The clinical picture of the nervous syndrome consists of combinations of spastic spinal paralysis and tabic symptoms (the so-called pseudotabes): spastic paraparesis with increased and pathological reflexes, clonuses, crawling, numbness of the extremities, girdle pain, violation of vibrational and deep sensitivity, sensory ataxia and dysfunction pelvic organs; less often - bulbar phenomena.

blood picture. The most characteristic symptom is anemia of the hyperchromic type. The morphological substrate of hyperchromia is large, hemoglobin-rich erythrocytes - macrocytes and megalocytes (the latter reach 12-14 microns or more). With an exacerbation of the disease, the number of reticulocytes in the blood decreases sharply. The appearance of a large number of reticulocytes portends a close remission.

An exacerbation of the disease is characterized by the appearance of degenerative forms of erythrocytes [poikilocytes, schizocytes, basophilic punctured erythrocytes, erythrocytes with Jolly bodies and Cabot rings (printing table, Fig. 3)], individual megaloblasts (printing table, Fig. 5). Changes in white blood are characterized by leukopenia due to a decrease in the number of cells of bone marrow origin - granulocytes. Among the cells of the neutrophilic series, giant, polysegmentonuclear neutrophils are found. Along with the shift of neutrophils to the right, there is a shift to the left with the appearance of young forms and even myelocytes. The number of platelets during the period of exacerbation is significantly reduced (up to 30,000 or less), however, thrombocytopenia, as a rule, is not accompanied by hemorrhagic phenomena.

Bone marrow hematopoiesis during the period of exacerbation of pernicious anemia occurs according to the megaloblastic type. Megaloblasts are a morphological expression of a kind of "dystrophy" of bone marrow cells in conditions of insufficient supply of a specific factor - vitamin B12. Under the influence of specific therapy, normoblastic hematopoiesis is restored (printing table, Fig. 6).

Symptoms of the disease develop gradually. For many years before the disease, gastric achilia is detected. At the beginning of the disease, general weakness is noted; patients complain of dizziness, palpitations at the slightest physical exertion. Then the dyspeptic phenomena, paresthesias join; patients go to the doctor, being already in a state of significant anemization. The course of the disease is characterized by cyclicity - a change in periods of improvement and deterioration. In the absence of proper treatment, relapses become longer and more severe. Before the introduction of liver therapy into practice, the disease fully justified its name "fatal" (pernicious). During the period of severe relapse - the sharpest anemization and the rapid progression of all the symptoms of the disease - a life-threatening coma (coma perniciosum) may develop.

Pathological anatomy. An autopsy of a person who died from pernicious anemia reveals a sharp anemia of all organs, with the exception of the red bone marrow; the latter, being in a state of hyperplasia, fills the diaphysis of the bones (printing table, Fig. 7). There is fatty infiltration of the myocardium ("tiger heart"), kidneys, liver; in the liver, spleen, bone marrow, lymph nodes - hemosiderosis (printing table, Fig. 8). Changes in the digestive organs are characteristic: the papillae of the tongue are atrophic, atrophy of the gastric mucosa and its glands - anadenia. In the posterior and lateral columns of the spinal cord, very characteristic degenerative changes are noted, referred to as combined sclerosis, or funicular myelosis.

Rice. 3. Blood in anemia: 1 - 4 - erythrocytes of the last stage of normal hematopoiesis (the transformation of an erythroblast into an erythrocyte); 5-9 - disintegration of the nucleus with the formation of Jolly bodies in basophilic punctured (5, 6) and polychromatophilic (7 - 9) erythrocytes; 10 and 11 - Jolly bodies in orthochromic erythrocytes; 12 - chromatin dust particles in erythrocytes; 13 - 16 - Kebot rings in basophilic punctured (13, 14) and orthochromic (15, 16) erythrocytes (pernicious anemia); 17 - 23 - basophilic punctured erythrocytes in lead anemia; 24 and 25 - polychromatophilic erythrocytes (microcyte and macrocyte); megalocyte (26) and poikilocyte (27) in pernicious anemia; 28 - normocyte; 29 - microcytes.

Rice. 5. Blood in pernicious anemia (severe relapse): orthochromic (1) and polychromatophilic (2) megalocytes, erythrocytes with Cabot rings (3), Jolly bodies (4) with basophilic puncture (5), megaloblasts (6), polysegmentonuclear neutrophil (7) , anisocytosis and poikilocytosis (8).

Rice. 6. Bone marrow in pernicious anemia (initial remission 24 hours after the administration of 30 μg of vitamin B12): 1 - normoblasts; 2 - metamyelocytes; 3 - stab neutrophil; 4 - erythrocyte.

Rice. 7. Myeloid hyperplasia of the bone marrow in malignant anemia.

Rice. eight. Hemosiderin pigmentation of the periphery of the hepatic lobules in pernicious anemia (reaction to Prussian blue).

Treatment. Since the 1920s, raw liver, especially lean veal liver, passed through a meat grinder (200 g per day) has been used with great success for the treatment of malignant anemia. A great achievement in the treatment of pernicious anemia was the manufacture of liver extracts, especially for parenteral administration (campolone, antianemin). The specificity of the action of hepatic drugs in pernicious anemia is due to their content of vitamin B12, which stimulates the normal maturation of erythroblasts in the bone marrow.

The greatest effect is achieved with the parenteral use of vitamin B12. The daily dose of vitamin B2 is 50-100 mcg. The drug is administered intramuscularly, depending on the patient's condition - daily or every 1-2 days. Oral use of vitamin B12 is effective only in combination with the simultaneous intake of an internal anti-anemic factor (gastromucoprotein). Currently, favorable results have been obtained from the treatment of patients with pernicious anemia through the internal use of the drug mucovit (available in the form of a dragee) containing vitamin B12 (200-500 mcg) in combination with gastromucoprotein (0.2). Mucovit is prescribed 3-6 tablets per day daily until the onset of a reticulocyte crisis and then 1-2 times a day until the onset of hematological remission.

The immediate effect of antianemic therapy in terms of replenishing the blood with newly formed erythrocytes begins to affect the rise of reticulocytes to 20-30% or more from the 5-6th day of treatment (“reticulocyte crisis”). Following the reticulocyte crisis, the amount of hemoglobin and erythrocytes begins to increase, which after 3-4 weeks reaches a normal level.

Folic acid, administered orally or parenterally at a dose of 30-60 mg or more (up to 120-150 mg) per day, causes a rapid onset of remission, but does not prevent the development of funicular myelosis. In funicular myelosis, vitamin B12 is used intramuscularly in large doses of 200-400 micrograms, in severe cases, 500-000 (!) micrograms per day] until complete clinical remission is achieved. The total dose of vitamin B12 during a 3-4-week course of anemia treatment is 500-1000 mcg, with funicular myelosis - up to 5000-10,000 mcg and above.

The effectiveness of vitamin B12 therapy has a known limit, upon reaching which the growth of quantitative blood parameters stops and anemia acquires a hypochromic character; during this period of the disease, it is advisable to use treatment with iron preparations (2-3 g per day, washed down with diluted hydrochloric acid).

The question of the use of blood transfusions in pernicious anemia in each case is decided according to indications. An unconditional indication is a pernicious coma, which poses a threat to life due to increasing hypoxemia. Repeated blood transfusions or (better) erythrocyte mass (250-300 ml each) often save the lives of patients until the moment when the therapeutic effect of vitamin B12 manifests itself.

Prevention. The minimum daily human need for vitamin B12 is 3-5 mcg, therefore, in order to prevent the recurrence of pernicious anemia, it can be recommended to inject 100-200 mcg of vitamin B12 2 times a month, and in spring and autumn (when relapses occur more often) - once a week or 10 days. It is necessary to systematically monitor the composition of the blood in persons who have undergone extensive resection of the stomach, as well as those who have persistent gastric achilia, provide them with a complete diet, and, if necessary, apply early antianemic treatment. It should be remembered that pernicious anemia can be an early symptom of stomach cancer. In general, it is known that patients with stomach achylia and especially pernicious anemia more often than others develop stomach cancer. Therefore, all patients with pernicious anemia should be under dispensary observation and annually undergo a control x-ray examination of the stomach.

- a violation of the red germ of hematopoiesis, due to a lack of cyanocobalamin (vitamin B12) in the body. With B12-deficiency anemia, circulatory-hypoxic (pallor, tachycardia, shortness of breath), gastroenterological (glossitis, stomatitis, hepatomegaly, gastroenterocolitis) and neurological syndromes (impaired sensitivity, polyneuritis, ataxia) develop. Confirmation of pernicious anemia is based on the results of laboratory tests (clinical and biochemical blood tests, bone marrow punctate). Treatment of pernicious anemia includes a balanced diet, intramuscular injection of cyanocobalamin.

ICD-10

D51.0 Vitamin B12 deficiency anemia due to intrinsic factor deficiency

General information

Pernicious anemia is a type of megaloblastic deficiency anemia that develops with insufficient endogenous intake or absorption of vitamin B12 in the body. "Pernicious" in Latin means "dangerous, disastrous"; in the domestic tradition, such anemia used to be called "malignant anemia". In modern hematology, pernicious anemia is also synonymous with B12-deficiency anemia, Addison-Birmer disease. The disease occurs more often in people older than 40-50 years, somewhat more often in women. The prevalence of pernicious anemia is 1%; however, about 10% of older people over the age of 70 suffer from vitamin B12 deficiency.

Causes of pernicious anemia

The daily human need for vitamin B12 is 1-5 micrograms. It is satisfied by the intake of the vitamin with food (meat, dairy products). In the stomach, under the action of enzymes, vitamin B12 is separated from dietary protein, but for absorption and absorption into the blood, it must combine with a glycoprotein (Castle factor) or other binding factors. The absorption of cyanocobalamin into the bloodstream occurs in the middle and lower parts of the ileum. The subsequent transport of vitamin B12 to tissues and hematopoietic cells is carried out by blood plasma proteins - transcobalamins 1, 2, 3.

The development of B12-deficiency anemia can be associated with two groups of factors: alimentary and endogenous. Nutritional causes are due to insufficient intake of vitamin B12 with food. This can occur with fasting, vegetarianism, and diets that exclude animal protein.

Under endogenous causes is meant a violation of the absorption of cyanocobalamin due to a deficiency of the internal factor of Castle with its sufficient intake from the outside. Such a mechanism for the development of pernicious anemia occurs in atrophic gastritis, a condition after gastrectomy, the formation of antibodies to the intrinsic factor of Castle or the parietal cells of the stomach, and congenital absence of the factor.

Violation of the absorption of cyanocobalamin in the intestine can be observed with enteritis, chronic pancreatitis, celiac disease, Crohn's disease, diverticula of the small intestine, tumors of the jejunum (carcinoma, lymphoma). Increased consumption of cyanocobalamin may be associated with helminthiases, in particular, diphyllobothriasis. There are genetic forms of pernicious anemia.

Vitamin B12 absorption is impaired in patients undergoing small bowel resection with gastrointestinal anastomosis. Pernicious anemia may be associated with chronic alcoholism, the use of certain drugs (colchicine, neomycin, oral contraceptives, etc.). Since the liver contains a sufficient reserve of cyanocobalamin (2.0-5.0 mg), pernicious anemia develops, as a rule, only 4-6 years after the violation of the intake or absorption of vitamin B12.

In conditions of vitamin B12 deficiency, there is a deficiency of its coenzyme forms - methylcobalamin (participates in the normal course of erythropoiesis processes) and 5-deoxyadenosylcobalamin (participates in metabolic processes occurring in the central nervous system and peripheral nervous system). The lack of methylcobalamin disrupts the synthesis of essential amino acids and nucleic acids, which leads to a disorder in the formation and maturation of red blood cells (megaloblastic type of hematopoiesis). They take the form of megaloblasts and megalocytes, which do not perform an oxygen transport function and are rapidly destroyed. In this regard, the number of erythrocytes in the peripheral blood is significantly reduced, which leads to the development of anemic syndrome.

On the other hand, with a deficiency of the coenzyme 5-deoxyadenosylcobalamin, the metabolism of fatty acids is disturbed, as a result of which toxic methylmalonic and propionic acids accumulate, which have a direct damaging effect on the neurons of the brain and spinal cord. In addition, myelin synthesis is disrupted, which is accompanied by degeneration of the myelin layer of nerve fibers - this is due to damage to the nervous system in pernicious anemia.

Symptoms of pernicious anemia

The severity of pernicious anemia is determined by the severity of circulatory-hypoxic (anemic), gastroenterological, neurological and hematological syndromes. Signs of an anemic syndrome are nonspecific and are a reflection of a violation of the oxygen transport function of erythrocytes. They are represented by weakness, decreased endurance, tachycardia and palpitations, dizziness and shortness of breath when moving, low-grade fever. On auscultation of the heart, a whirlpool or systolic (anemic) murmur may be heard. Outwardly, there is pallor of the skin with a subicteric shade, puffiness of the face. A long "experience" of pernicious anemia can lead to the development of myocardial dystrophy and heart failure.

Gastroenterological manifestations of B12-deficiency anemia are decreased appetite, stool instability, hepatomegaly (fatty liver). The classic symptom found in pernicious anemia is a raspberry-colored “varnished” tongue. The phenomena of angular stomatitis and glossitis, burning and pain in the tongue are characteristic. During gastroscopy, atrophic changes in the gastric mucosa are detected, which are confirmed by endoscopic biopsy. Gastric secretion is sharply reduced.

Neurological manifestations of pernicious anemia are caused by damage to neurons and pathways. Patients indicate numbness and stiffness of the limbs, muscle weakness, impaired gait. Possible incontinence of urine and feces, the occurrence of persistent paraparesis of the lower extremities. An examination by a neurologist reveals a violation of sensitivity (pain, tactile, vibration), increased tendon reflexes, symptoms of Romberg and Babinsky, signs of peripheral polyneuropathy and funicular myelosis. With B12-deficiency anemia, mental disorders can develop - insomnia, depression, psychosis, hallucinations, dementia.

Diagnosis of pernicious anemia

In order to compensate for the deficiency of cyanocobalamin, intramuscular injections of vitamin B12 are prescribed. Correction of the conditions that led to B12-deficiency anemia (deworming, taking enzyme preparations, surgical treatment) is required, and with the alimentary nature of the disease, a diet with a high content of animal protein. In case of violation of the production of the intrinsic factor of Castle, glucocorticoids are prescribed. Blood transfusions are used only for severe anemia or signs of anemic coma.

Against the background of therapy for pernicious anemia, blood counts usually normalize after 1.5-2 months. The neurological manifestations persist the longest (up to 6 months), and with late treatment they become irreversible.

Prevention of pernicious anemia

The first step to prevent pernicious anemia should be a nutritious diet that provides sufficient intake of vitamin B12 (meat, eggs, liver, fish, dairy products, soy). Timely therapy of pathologies of the gastrointestinal tract that violates the absorption of the vitamin is necessary. After surgical interventions (resection of the stomach or intestines), it is necessary to conduct maintenance courses of vitamin therapy.

Patients with B12-deficiency anemia are at risk for the development of diffuse toxic goiter and myxedema, as well as stomach cancer, therefore, they need to be monitored by an endocrinologist and gastroenterologist.

Pernicious anemia (B12 deficiency, megaloblastic or Addison-Birmer disease) is a disease of the blood system characterized by a decrease in hemoglobin and the number of red blood cells, resulting from a lack of vitamin B12 (cyanocobalamin) and affecting the digestive system, nervous and hematopoietic systems.

What happens in pernicious anemia?

Normally, vitamin B12 is absorbed in the stomach when it is separated from the proteins that come with food (with meat, dairy products). This breakdown requires gastric enzymes and the specific intrinsic factor of Castle, which also serves as a carrier protein for vitamin B12. Only in the presence of this factor, the vitamin is absorbed into the bloodstream; in its absence, cyanocobalamin enters the large intestine and is excreted from the body along with feces.

Vitamin deficiency is not clinically manifested immediately, since it is synthesized in the liver and for some time (about 2-4 years) hypovitaminosis is compensated. Castle factor is produced by specific parietal cells of the gastric mucosa, and if they are damaged or destroyed, the risk of pernicious anemia increases.

Possible causes of pernicious anemia

How does pernicious anemia manifest itself?

Pernicious anemia is a polysyndromic disease, that is, it manifests itself in many symptoms. Vitamin B12 is involved in the formation of blood cells, in metabolic processes occurring in the nervous system. Therefore, the lack of this vitamin is primarily manifested by symptoms from the blood system, the organs of the gastrointestinal tract and the nervous system.

1. anemic syndrome. With a lack of cyanocobalamin, the formation of normal red blood cells is disrupted, they cease to carry oxygen to tissues and organs. Because of this, weakness, pallor of the skin, fatigue, tachycardia (increased heart rate), shortness of breath and dizziness occur. Sometimes subfebrile condition may occur - an increase in body temperature to low numbers (not higher than 38 degrees).
2. Gastroenterological syndrome - manifestations of the organs of the digestive system. There is a decrease in appetite, a violation of the stool (constipation or diarrhea), an increase in the size of the liver (hepatomegaly). Language changes are characteristic. They manifest themselves in the form of inflammatory reactions of the mucous membrane of the tongue (glossitis) or the corners of the lips (angulitis), in the form of burning and pain in the tongue. Also a specific symptom will be "lacquered tongue" - this is a smooth crimson tongue. In the stomach, atrophy of the mucous membrane and the development of atrophic gastritis with a decrease in secretory functions will occur.


3. A neurological syndrome is a manifestation of the nervous system. They arise as a result of a violation of the metabolism of fats and the formation of toxic acids that affect nerve cells. There is also a violation of the synthesis of myelin, which is necessary for the formation of a protective sheath of nerves. The syndrome manifests itself in the form of numbness of the limbs, impaired gait and fine motor skills, stiffness in the muscles. Also, as a result of relaxation of the sphincters, enuresis (urinary incontinence) and encopresis (fecal incontinence) can occur. Psychiatric symptoms such as insomnia, depression, psychosis, or hallucinations may occur.
4. Hematological syndrome - symptoms from the blood. It manifests itself in the results of a blood test in the form of progressive anemia (decrease in hemoglobin and erythrocytes), leukopenia (decrease in the number of leukocytes), the appearance of atypical erythrocytes - megaloblastic forms.

Etiology

Three factors are involved in the development: PAa) family predisposition, b) severe atrophic gastritis, c) association with autoimmune processes.

In the UK, familial predisposition to PA was noted in 19% of patients, and in Denmark - in 30%. The average age of patients is 51 years in the group with a family predisposition and 66 years in the group without a family predisposition. In identical twins, PA occurred at about the same time. Research by Callender, Denborough (1957)

showed that 25% of relatives of patients with PA suffer from achlorhydria, and in a third of relatives with achlorhydria (8% of the total) the content of vitamin B12 in serum is reduced and its absorption is impaired. There is a connection between blood type A, on the one hand, and PA and gastric cancer, on the other hand, there is no clear connection with the HLA system.

More than 100 years have passed since Fenwick (1870) found atrophy of the gastric mucosa and cessation of pepsinogen production in patients with PA. Achlorhydria and the virtual absence of intrinsic factor in gastric juice are characteristic of all patients. Both substances are produced by the parietal cells of the stomach. Mucosal atrophy involves the proximal two-thirds of the stomach. Most or all of the secreting cells die and are replaced by mucus-forming cells, sometimes of the intestinal type. Lymphocytic and plasmacytic infiltration is observed. Such a picture, however, is typical not only for PA. It is also found in simple atrophic gastritis in patients without hematological abnormalities, and they do not develop PA even after 20 years of observation.

The third etiological factor is represented by the immune component. Two types of autoantibodies have been found in PA patients:

parietal cells and intrinsic factor.

The method of immunofluorescence in the serum of 80-90% of patients with PA reveals antibodies that react with the parietal cells of the stomach.

the same antibodies are present in the serum of 5-10% of healthy individuals. In elderly women, the frequency of detection of antibodies to the parietal cells of the stomach reaches 16%. Microscopic examination of biopsies of the gastric mucosa in almost all individuals with serum antibodies to the parietal cells of the stomach reveals gastritis. The introduction of antibodies to the parietal cells of the stomach in rats leads to the development of moderate atrophic changes, a significant decrease in the secretion of acid and intrinsic factor. These antibodies obviously play an important role in the development of atrophy of the gastric mucosa.

Antibodies to intrinsic factor are present in the serum of 57% of patients with PA and are rarely found in individuals who do not suffer from this disease. When administered orally, antibodies to intrinsic factor inhibit the absorption of vitamin B12 due to their association with intrinsic factor, which prevents the latter from binding to vitamin B!2.

IgG. In some patients, antibodies are present only in gastric juice. Based on the detection of antibodies in both serum and gastric juice, it can be concluded that such antibodies to intrinsic factor are detected in approximately 76% of patients.

Another form of immune response to intrinsic factor is cellular immunity as measured by inhibition of leukocyte migration or lymphocyte blast transformation. Cellular immunity is found in 86% of patients. If we combine the results of all tests, i.e. data on the presence of humoral antibodies in serum, in gastric secretions, immune complexes in gastric secretions and

Pernicious anemia is an anemia that occurs due to a lack of such an important vitamin as B12. The deficiency itself can be caused by the fact that a person does not eat right, or his body has simply lost the ability to absorb it. Often this disease is inherited.

It is worth recalling that anemia is nothing more than a disease that is associated with a lack of red blood cells (erythrocytes). With it, the blood becomes unable to deliver as much oxygen to the tissue cells as they need. The consequences of anemia are different. The main and universal, perhaps, is chronic fatigue.

Pernicious anemia can cause the cells to simply stop dividing, or become really big enough that they can't leave the bone marrow.

Vitamin B12 in our body for the reason that it is he who is responsible for the formation of red blood cells. It also has a positive effect on the functioning of the nervous system. As a rule, a person receives it from meat, fish, eggs and various dairy products. Not infrequently, the body lacks it, not for the reason that a person does not consume the necessary foods, but because the stomach lacks protein, without which B12 cannot be absorbed.

By the way, a curious fact is that pernicious anemia often torments those who have chosen the wrong diet to fight excess weight. I think everyone understands what is at stake.

Pernicious anemia: symptoms

It develops very slowly, and its first symptoms usually appear when the human body is weakened (for example, after suffering an infectious disease). Most of all, people from thirty-five to sixty years old are susceptible to it. Gender doesn't matter.

Symptoms can be identified as follows:

- burning tongue;

- the patient constantly feels tingling in the fingers;

- chronic fatigue;

- there are muscle pains;

- appetite decreases;

- Appears, diarrhea, vomiting, belching. Flatulence is also quite possible;

- the skin loses its sensitivity.

It is also worth mentioning that the tongue becomes a bright red color.

This disease always progresses in the spring.

Pernicious anemia may well lead to the pathology of the nervous system, heart, and much more. All this means that treatment should be started immediately after the disease has been diagnosed. The diagnosis, as a rule, is made by the doctor after he listens to all the patient's complaints and checks the test results. Anemia is not always difficult to detect in the early stages, which means that a lot depends on your attending physicians in this case.

Pernicious anemia: treatment and prevention of the disease

All treatment usually comes down to the fact that the patient is given B12 in certain doses. Unfortunately, some people will have to take it for the rest of their days on earth.

During treatment, doctors try to make up for the lack of the right vitamin, eliminate the consequences of this disease, and also eliminate the cause (if pernicious anemia was not caused by malnutrition).

I would like to note that vitamin B12 can be injected with a syringe or taken in the form of tablets. In mild cases, already a few days after the start of the reception, the first improvements will appear, and after one to two weeks the person will forget that he had this ailment.

Regarding prevention, it is worth saying that you need to eat right, and not just anyhow. Diet is a good thing, however, you should not take it from a source that raises at least some doubt. Ask your friends, consult a dietitian - you should not rush, as it is much easier to undermine health than to restore it.

Of course, you also need to use all kinds of vitamin complexes, which contain a lot of B12.

B12 deficiency anemia

A disease like pernicious anemia has a strong similarity with the usual anemia caused by a lack of vitamin B12. Both diseases are characterized by neurological manifestations and megaloblastic process of hematopoiesis.

Pernicious anemia (from Latin perniciosus - fatal, dangerous) or B12-deficiency anemia or megaloblastic anemia or Addison-Birmer disease or (obsolete name) malignant anemia - a disease caused by impaired hematopoiesis due to a lack of vitamin B12 in the body. The bone marrow and tissues of the nervous system are especially sensitive to the deficiency of this vitamin.

The specificity of this disease is the mandatory presence of three constituent causes:

• aggravated stage of atrophic gastritis;
• deficiency of vitamin B12, resulting from the difficulty of assimilation, regardless of the quantitative composition in food;
• the transformation of hematopoiesis into a megaloblastic type, which is typical for the embryonic development of the human body.

The second name of this pathology is pernicious or benign anemia, occurring in 1-2%, mainly after the age of sixty. Women are more commonly affected by this disease than men. In the case when there is a genetic predisposition, then people at a young and middle age can get sick.

Causes of pernicious anemia

The first description of the symptoms and causes of pernicious anemia was presented in the second half of the nineteenth century by the English physician Addison, and the second version was described by Birmer thirteen years later. The opinion of both doctors was that the disease could not be cured because the underlying causes had not been identified.

In 1870, the relationship of anemia with atrophy of the gastric mucosa and with the interruption of the production of pesinogen was proved.

Further study of the disease made it possible to determine the characteristic features and the degree of modifications in the stomach. It turned out that the necrosis of parietal cells occurs in most of the mucosa and, along with this, the possibility of producing the enzyme is lost. Dead cells are replaced by others, similar to intestinal mucus production.

The walls of the digestive organ are infiltrated by lymphocytes and plasma. Such changes occur not only with pernicious anemia, but also with atrophic gastritis, and even with a long course of the disease, anemia is not observed.

Studies have shown that the presence of two more factors is also necessary for the occurrence of pernicious anemia:

• genetic predisposition.
• Autoimmune state of the body.

The impact of immunity has been proven by the method of immunofluorescence of 2 types of antibodies in the blood serum of patients:

1. in 90% of patients to parietal cells;
2. in 57% of patients to the internal factor that produces the enzyme.

Basically, antibodies are also detected in gastric juice, which inhibit the absorption of vitamin B12.

Modern knowledge about immunity makes it possible to get a more complete picture of the causes of the disease. The genetic factor is confirmed by the presence of antibodies in healthy relatives. When the functioning of the immune system is impaired, antibodies appear against cells in the pancreas and thyroid gland, as well as the adrenal glands.

To determine which manifestation is primary, a disease of the digestive organ or autoimmune processes, has not yet been clarified. Both symptoms are aggravated at the expense of each other and make it difficult to restore the mucosa.

The appearance of a lack of vitamin B12 occurs gradually as parietal cells shrink. In some cases, signs of anemia appear after five or more years.

Violation of hematopoiesis

The clinic of Addison-Birmer disease consists in the manifestation of general symptoms of anemia in combination with digestive and neurological disorders. These signs include:

• severe fatigue, lethargy;
• feeling of heaviness after eating in the epigastric region;
• pain in the mouth and tongue;
• feeling of numbness of the skin of the lower and upper extremities;
• slight weight loss, but mostly a tendency to be overweight;
• in the acute period, a disruption in the functioning of the intestine and a change in the color of the feces are likely;
• an increase in body temperature due to the accelerated breakdown of red blood cells.

There may also be other symptoms, but they are rare. These include:

1. deterioration of potency in men and impaired urination;
2. change in gait;
3. vision dysfunction.

With damage to the visual, olfactory and auditory nerve fibers, there is a violation of vision, smell and hearing. Very rarely, mental disorders and hallucinations were recorded.

Diagnosis of B12-deficiency anemia

At a doctor's appointment in a patient, after a thorough diagnosis of B12 deficiency anemia, it can be found:

• pallor of the skin;
• swelling of the face;
• brown pigment spots in the nose and cheekbones;
• insignificance of hysteria of the sclera.

When examining the oral cavity, the following symptoms are revealed:

1. in the initial stage of the disease, painful lesions appear in the form of cracks;
2. the occurrence of an inflammatory process with ulcers on the gums and mucous membranes, and then spreading to the pharynx and esophagus;
3. in the acute period of the disease, the tongue acquires a scarlet color due to atrophy and swelling of the folds.

When probing the abdomen, the soft part of the liver is determined with an increase in size. Changes in the spleen are extremely rare.

What additional testing is needed?

The diversity of the disease requires confirmation of the diagnosis by various specialists:

1. An examination by a neurologist helps to establish the lost sensitivity of the legs and arms, impaired motor functions, which indicate damage to the myelin sheaths in the spinal cord.
2. To rule out the possibility of a malignant formation, an endoscopy of the stomach should be performed. The presence of atrophic gastritis is characterized by the appearance of shiny zones where cell death occurs.
3. Conducting fluoroscopy shows increased evacuation and alignment of folds of the mucosa.
4. To prove the low absorption of vitamin B12 in the stomach, a Schilling diagnosis is made, in which the amount of the isolated element after administration is determined. A check is also made to see if the reduction in blood and urine levels is consistent. A low indicator indicates a large amount of antibodies in the gastric juice.
   5. In the study of gastric juice, low saturation with hydrochloric acid or its complete absence and a large amount of mucus are found.
   6. Histology of the mucosa shows thinning and disappearance of the glandular epithelium. Pernicious anemia is characterized by the absence of gastromucoprotein and acid secretion.
   7. The cardiogram reveals oxygen starvation of the myocardium. The presence of tachycardia contributes to the development of arrhythmia, ischemia and early cardiosclerosis.

Blood picture with vitamin B12 deficiency

With a shortage of 12

Vitamin B12 deficiency can lead to impaired hematopoiesis in the bone marrow. There is a decrease in hemoglobin, while the fall in the number of red blood cells is even greater.

Peripheral blood analysis reveals the presence of large cells of megalocytes and macrocytes, which are twice the size of an erythrocyte. Probably the appearance of cells with a modified structure and the remains of nuclei.

There are also changes in the following indicators:

• the quantitative composition of reticulocytes decreases;
• the growth of white blood is inhibited, which contributes to the formation of leukopenia and relative lymphocytosis;
• the number of platelets decreases, but growth in size is activated;
• Blast cells of the erythrocyte series of different maturity predominate in the bone marrow.

During remission, bone marrow hematopoiesis returns to normal.

Treatment for pernicious anemia

The main direction in the treatment of this disease is to eliminate the lack of vitamin B12. For this, the drug Oxycobalamin is prescribed, which is considered the most effective in the first stage of treatment.

If after six months there is a lack of iron, then iron-containing preparations in tablets are prescribed.

As a result of treatment, the number of reticulocytes increases, well-being improves, dizziness disappears, strength appears in the limbs. Stabilization will take at least a month and a half.

Features of the flow B 12 -deficiency anemia

Difficulties in diagnosis arise when clinical symptoms are present without changes in hematopoiesis. In this case, the diagnosis is atrophic gastritis, and not Addison-Birmer disease.

Fluctuations in the correctness of the diagnosis appear with a negative reaction to the introduction of vitamin B12 and the absence of neurological signs. It is also worth considering that the effectiveness of treatment can be eliminated by the diseases already present in the patient:

• pyelonephritis in a chronic form;
• kidney dysfunction;
• the presence of infection in the body for a long time;
• thyroid disease;
• malignant formations.

Forecast

Currently, it is rare to see an advanced form of pernicious anemia, this is possible only in the absence of medical therapy. At the moment, the disease is curable, but requires medical supervision and prevention of remission.

With a long course of the disease, patients develop concomitant pathology:

1. toxic diffuse thyroid disease is found in 1.8% of patients;
2. endocrine disease myxedema is found in 2.4% of cases.

The primary symptoms of cancer are:

• nonspecific weight loss;
• resistance to the drug cyanocobalamin;
• restoration of hematopoiesis with existing symptoms.

Incomplete study of the causes and consequences of Addison-Birmer anemia is a reason for the patient to take an active position in preventive actions against relapses. To do this, you should eat well and properly, give up bad habits and monitor your health.