Swelling of the feet in case of anemia
Malignant anemia (synonym: pernicious anemia, Addison-Birmer disease) - endogenous B12-avitaminosis. caused by atrophy of the glands of the fundus of the stomach, producing gastromukoprotein. This leads to a violation of absorption of vitamin B12, which is necessary for normal hematopoiesis. and the development of pathological megaloblastic hematopoiesis, resulting in anemia of the "pernicious" type. People are sick after the age of 50.
Characteristic violations of the cardiovascular,nervous, digestive and hematopoietic systems. Complaints of patients are diverse: general weakness, dyspnea. palpitation. pain in the region of the heart, swelling of the legs, a sensation of creeping crawling in the hands and feet, gait disorder, burning pain in the tongue, periodic diarrhea. Appearance of the patient is characterized by pale skin with a lemon-yellow hue. The scleras are subiclinic. Patients are not depleted. In the study of the cardiovascular system, anemic noise is typical, associated with a decrease in blood viscosity and acceleration of blood flow. On the part of the digestive organs, the so-called Hunter's glossitis is found (the tongue is bright red, the papillae smoothened), histamine-resistant achilias (lack of free hydrochloric acid and pepsin in the gastric contents). The liver and spleen are enlarged. With a significant reduction in the number of erythrocytes (below 2 million), 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).
Blood picture: anemia of hyperchromatic type, macrocytes, megalocytes, erythrocytes with Jolly bodies, Keboat rings, leukopenia, thrombocytopenia (during exacerbation).
Treatment is carried out with vitamin B12 -100-200 μgintramuscularly daily or a day before the onset of remission. When there is anemic coma - urgent hospitalization, blood transfusion. better erythrocytic mass (150-200 ml). To prevent recurrence, maintenance therapy with vitamin B12 is necessary. A systematic observation of the composition of blood in people with persistent a achillia, as well as those who underwent gastrectomy, was shown. Patients suffering from pernicious anemia, should be under clinical observation (possibly the 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 as endogenous B12-avitaminosis due to atrophy of the base glands producing gastromucoprotein, as a result of which the uptake of vitamin B1a necessary for normal, normoblastic, hematopoiesis is impaired, and develops pathological, megaloblastic, hematopoiesis, leading to anemia of the "pernicious" type.
Clinical picture. People who are older than 40-45 years are ill. Characteristic violations of the cardiovascular, nervous, digestive and hematopoietic systems. Complaints of patients are diverse: general weakness, shortness of breath, palpitation, pain in the heart, swelling of the legs, dizziness, a sensation of crawling in the hands and feet, gait disorder, burning pains in the tongue and esophagus, periodic diarrhea. Appearance of the patient is characterized by pale skin with a lemon-yellow hue. The scleras are subiclinic. Patients are not depleted. The face is puffy, puffiness in the ankles and feet. Edema can reach high degrees and is accompanied by ascites, hydrothorax. From the side of the cardiovascular system - the appearance of systolic noise on all the 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 acceleration of blood flow; angina of anoxemic nature is possible. With prolonged anemia, the fatty degeneration of organs develops, including the heart ("tiger heart"), as a result of persistent anoxemia. On the part of the digestive system - the so-called hunterovskogo (gunterovskogo) glossitis, the language is clean, bright red color, devoid of papillae. The analysis of gastric juice, as a rule, reveals histamine-resistant achilles. 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 erythrocytes (below 2 000 000), a fever of the wrong type is observed. Changes from 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 nervous syndrome consists of the combination of spastic spinal paralysis and tabicheskih symptoms (called psevdotabes): spastic paraparesis with increased and abnormal reflexes, clonus, feeling pins and needles, numbness, girdle pain, impaired vibration and deep sensitivity, sensory ataxia and disorder functions pelvic organs; less common bulbar phenomena.
Picture of blood. The most characteristic sign is anemiahyperchromic type. Morphological substrate of hyperchromia - large, hemoglobin-rich erythrocytes - macro-cells and megalocytes (the latter reach 12-14 microns and more). When the disease worsens, the amount of reticulocytes in the blood decreases sharply. The appearance of a large number of reticulocytes presages a close remission.
To exacerbate the disease is typicaldegenerative forms of erythrocytes (poikilocytes, schizocytes, basophil-punctured erythrocytes, erythrocytes with Jolly bodies and Keboat rings (color table in Figure 3)], individual megaloblasts (color table in Figure 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, polyspecific-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 in the period of exacerbation is significantly reduced (up to 30 000 and less), however, thrombocytopenia, as a rule, is not accompanied by hemorrhagic phenomena.
Bone marrow hemopoiesis in the period of exacerbationpernicious anemia is carried out by megaloblastic type. Megaloblasts are a morphological expression of a kind of "dystrophy" of bone marrow cells in conditions of insufficient transport of a specific factor - vitamin B12. Under the influence of specific therapy, normoblastic hematopoiesis is restored (color table in Figure 6).
Symptoms of the disease develop gradually. Many years before the disease, gastric achilles are found. At the beginning of the disease, general weakness is noted; patients complain of dizziness, palpitation at the slightest physical strain. Then dyspeptic phenomena, paresthesia, are added; patients turn to the doctor, being already in a state of significant anemization. The course of the disease is characterized by a cycle - a change in periods of improvement and deterioration. In the absence of proper treatment, relapses become more and more prolonged and severe. Prior to the introduction into practice of hepatic therapy, the disease fully justified its name as "fatal" (pernicious). In a period of severe recurrence - severe anemia and rapid progression of all symptoms of the disease - a life-threatening coma (coma perniciosum) can develop.
Pathological anatomy. At autopsy of the deceased person from pernicious anemia, an abnormal anemia of all organs is detected, except for the red bone marrow; the latter, being in a state of hyperplasia, fills in the diaphysis of bones (color table in Figure 7). There is a fatty infiltration of the myocardium ("tiger heart"), kidneys, liver; in the liver, spleen, bone marrow, lymph nodes - hemosiderosis (color table in Figure 8). The changes on the part of the digestive organs are characteristic: the papillae of the tongue are atrophic, the atrophy of the mucous membrane of the stomach and its glands is anadenia. In the posterior and lateral columns of the spinal cord, there are very characteristic degenerative changes, referred to as combined sclerosis, or funicular myelosis.
Fig. 3. Blood in anemia: 1 - 4 - erythrocytes of the last stage of normal hematopoiesis (transformation of erythroblast into erythrocyte); 5 -9 - decay of the nucleus with the formation of Jolly bodies in basophilically punctured (5, 6) and polychromatophilic (7-9) erythrocytes; 10 and 11 - Jolly's bodies in the orthochromic red blood cells; 12 - chromatinous motes in erythrocytes; 13 - 16 - Quota rings in basophilically punctured (13, 14) and orthochromic (15, 16) erythrocytes (pernicious anemia); 17 - 23 - basophilic punctured erythrocytes in lead anemia; 24 and 25 - polychromatophilic erythrocytes (microcyt and macrocyte); megalocyte (26) and poikilocyte (27) with pernicious anemia; 28 - normocyte; 29 - microcytomas.
Fig. 5. Blood with pernicious anemia (severe relapse): megalocytes orthochromic (1) and polychromatophilic (2), erythrocytes with Keboat rings (3), Jolly bodies (4) with basophilic position (5), megaloblasts (6), polysegmentonyuclear neutrophils (7), anisocytosis and poikilocytosis (8).
Fig. 6. Bone marrow with pernicious anemia (initial remission 24 hours after the administration of 30 μg of vitamin B12): 1 - normoblasts; 2 - metamyelocytes; 3 - stab neutrophil; 4 - erythrocyte.
Fig. 7. Myeloid bone marrow hyperplasia in malignant anemia.
Fig. 8. Hemosiderinovaya pigmentation of the periphery of the hepatic lobules with pernicious anemia (reaction to the Berlin azure).
Treatment. Since the 20-ies for the treatment of malignantanemia with great success is applied to the raw liver, especially low-fat veal, passed through a meat grinder (200 g per day). A great achievement in the therapy of pernicious anemia was the production of hepatic extracts, especially for parenteral administration (campolon, anti-anemine). Specificity of the action of hepatic drugs in pernicious anemia is due to the content of vitamin B12 in them, which stimulates the normal maturation of erythroblasts in the bone marrow.
The greatest effect is achieved with parenteralapplication of vitamin B12. The daily dose of vitamin В2-50-100 mcg. The drug is administered intramuscularly depending on the patient's condition - every day or 1-2 days. Oral administration of vitamin B12 is effective only in combination with the simultaneous administration of an internal anti-anemic factor (gastromucoprotein). Currently, favorable results have been obtained from the treatment of patients with pernicious A. by internal application of the drug mucovita (available as a dragee) containing vitamin B12 (200-500 μg each) in combination with gastromucoprotein (0.2). Mucovitis is prescribed for 3-6 tablets daily before the onset of the reticulocytic crisis and 1-2 times a day before the onset of hematologic remission.
The immediate effect of anti-anemic therapy inthe sense of blood replenishment with newly formed red blood cells begins to affect from the 5th-6th day of treatment to a rise of reticulocytes to 20-30% and higher ("reticulocyte crisis"). Following the reticulocytic crisis, the amount of hemoglobin and erythrocytes begins to increase, which reaches a normal level 3-4 weeks later.
Folic acid administered orallyparenterally in a dose of 30-60 mg or more (up to 120-150 mg) per day, causes a rapid onset of remission, but it does not prevent the development of funicular myelosis. With funicular myelosis, vitamin B12 is used intramuscularly at large doses of 200-400 μg, in severe cases, 500,000 (!) Μg per day] until a complete clinical remission is achieved. The total dose of vitamin B12 during a 3-4-week course of treatment for anemia is 500-1000 μg, with funicular myelosis - up to 5000-10,000 μg and higher.
The effectiveness of vitamin B12 therapy hasa known limit, upon which the growth of quantitative indicators of blood ceases and anemia acquires a hypochromic character; In this period of the disease, it is advisable to apply the treatment with iron preparations (2-3 g per day, washed down with diluted hydrochloric acid).
The question of the use of blood transfusions forpernicious A. in each case is decided by the indications. An unconditional indication is a pernicious coma, which poses a threat to life as a result of increasing hypoxemia. Repeated blood transfusions or (better) erythrocyte mass (250-300 ml) often save the life of patients until the moment when the therapeutic effect of vitamin B12.
Prevention. The minimum daily human need forvitamin B12 is 3-5 μg, therefore, in order to prevent recurrence of pernicious anemia, it can be recommended to inject 100-200 μg of vitamin B12 in injections 2 times a month, and in spring and autumn (when relapses occur more often) - once a week or 10 days. It is necessary to monitor systematically the composition of blood in individuals who have undergone extensive gastrectomy, as well as those with persistent stomach ailments, provide them with a full-fledged diet, and if necessary, apply early anti-anemis treatment. It should be remembered that pernicious A. may be an early symptom of stomach cancer. In general, it is known that patients with stomach aholy and especially pernicious anemia most often get stomach cancer. Therefore, all patients with pernicious A. should be under clinical supervision and annually undergoing a controlled radiographic examination of the stomach.