Congenital Hemolytic Anemia
(Internal Disease - Circulatory)
Thalassemia
Etiology and pathogenesis. The disease is inherited family trait the predominance of red blood cells of fetal embryonic hemoglobin.
Symptoms and flow. There are large, small and minimal thalassemia. The first form of thalassemia in children-homozygotes, the latter two forms a child of heterozygotes. A typical symptom for thalassemia major is a progressive anemia with erythroblastosis, enlarged spleen and liver, osteoporosis, with peculiar changes in bones of the skull (disproportionate proliferation of parietal and occiput) as a consequence of abnormal proliferation of erythroblastic germ in the bone marrow. In the blood, anemia with eritroblastos, the presence of peculiar some special erythrocytes with increasing maximum and minimum simultaneously with a decrease in osmotic resistance.
In bone marrow at the background of general hyperplasia of cellular elements show an increase in red growth and a slight increase in the number of reticular cells. In the liver and spleen are determined extramedullary foci of erythropoiesis and leucopoiesis.
Treatment. In order to avoid the development of hemosiderosis hematherapy shown only when hemolytic crisis. Steroid hormones give temporary success. Splenectomy is effective not in all cases, and after the operation in the blood remain some special erythrocytes.
Sickle Cell Anemia
Etiology. The disease is congenital inferiority of red blood cells associated with the presence in the past to 60-90% of pathological hemoglobin S. Develops in children with homozygotes. Children heterozygote, inherited trait camber from one of the parents are carriers of sickle-cell abnormalities. Symptom of camber in red blood cells due to low solubility of hemoglobin S and its crystallization. Sickle-shaped red blood cells differ increased resistance to hypotonic solutions of sodium chloride.
Symptoms are similar to the pattern of congenital microspherocytic hemolytic anemia. Observed congenital defects of bones: skull tower, high sky. There is a tendency to osteoporosis in the bones, in the bones of the skull revealed subulate appendages. In the blood, anemia, bilirubinemia, increased urobilin in urine and stercobilin in feces. The disease is characterized by an undulating course, hemolytic crises are replaced by remissions. In the gallbladder pigment stones. In many cases, developed splenomegaly. A number of patients in the period of increased hemolysis appears a tendency to thrombosis of small vessels of various organs.
Recognition is based on the type of hemolytic anemia with the presence of peculiar crescent-shaped red blood cells and the application of special tests on the camber of red blood cells (with metabilsulfitom).
Treatment. During the period of anemia required blood transfusions. In marked splenomegaly, and especially the development of the spleen infarctions shown splenectomy.
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