Hereditary hemolytic anemia - Causes,Symptoms,Diagnosis,Treatment,Analysis,disease

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Saturday, 25.03.2017, 00:51
Main » Pediatrics » Hereditary hemolytic anemia 
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Hereditary hemolytic anemia


Hereditary hemolytic anemia

Microspherocytic hemolytic anemia (anemia of Minkowski-Chauffard)


At the heart disease is genetically caused anomaly structure of erythrocyte membranes, which leads to the formation of spherocytes, which are subjected to the enhanced destruction mainly in the spleen, resulting in anemia, jaundice, splenomegaly.

Mostly sharp jaundiced skin and sclera, the change of bone ("tower skull", "sky high"), splenomegaly. If the blood test reveals anemia, high reticulocytosis. The analysis of urine - urobilinuria. In the study of bone marrow revealed a sharp increase in erythropoiesis.

Undulating course of the disease, triggered by increased hemolysis, as a rule, co-morbidities. Frequent hemolytic crises may lead to depletion of the compensatory capacity of bone marrow. Radical and effective 100% of the treatment is splenectomy.

Macrocytic hemolytic anemia

Dominantly inherited disease caused by deficiency of red blood cells (violation of various enzyme systems), leading to rapid destruction of the latter. Hemolytic crises are most likely to be provoked drugs. The clinical picture is similar to that in mikrotsetarnoy hemolytic anemia.

Hemoglobinopathies (thalassemia)

Inherited in a dominant and recessive manner. And each of these options is characterized by the presence of fetal hemoglobin. The most severe disease is considered to be a big thalassemia, which is expressed already in the neonatal period and ending fatally. Asymptomatic forms are due to the presence of HbA, HbF, and others formed early appearance of the child: "tower skull", wide-set eyes, broad flat nose. There is an increase in the abdomen as a result of increased parenchymal organs, primarily the spleen.



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