Anemia Hemolytic Nonspherocytic Hereditary

Anemia Hemolysis (hemolytic) Nespherocyte (non-spherocytic) (haemolytic anemia; a. haemolytic): a hereditary hematological disease characterized by an increase in the number of damaged red blood cells, which carry out in the blood increased production of indirect hemoglobin or hemosiderin, which cannot

Hereditary hemolytic nonspherocytic anemia (AHN) is a genetically determined multifactorial disease of hemostasis. According to a number of authors (Zarubina E.Yu.), it prevails in males (about 46%) in combination with anhidrotic ectodermal dysplasia (12%) [2]. The hemolytic state of the blood is explained by the fact that, apparently, red blood cells have a defect in protection (membrane defects), which leads to their rapid destruction in the spleen, discoloring the blood reddish-brown and the accumulation of bilirubin and indirect bilirubin in the blood. Moreover, even in the spleen, the spleen cannot stabilize free hemoglobin. The etiology and pathogenesis of this disease are not fully understood. Researchers have long debated whether it is an inherited disease.

Hereditary hemolytic anemia Nonspherocytic Hereditary nispherocytic type is a rare form of hereditary hemolytic anemia caused by nonspherocytic erythrocytes due to various immature anomalies of the erythroid lineage of the bone marrow. The genetic cause is mutations in genes encoding the synthesis or delivery of 3-thiazinone. Most often, the disease occurs in familial cases of the disease, inherited in an autosomal recessive manner and caused by pathology of the genes of the folate cycle enzyme system, as a result of impaired DNA synthesis, hemoglobin functioning and impaired oxidative properties and melanin formation of iron. There is evidence that damage to the IIC genes on the X and Y chromosomes may also be a source of hereditary nispherocytosis.