Features of hematopoietic regenerative function in hereditary microspherocytosis (Minkowski-Shoffar disease) with early manifestation

Hereditary spherocytosis is one of the most common hereditary hemolytic anemias, the pathogenetic link of which is erythrocyte membranopathy. The disease is heterogeneous in the severity of clinical manifestations and varies from asymptomatic forms to frequent severe hemolytic crises. Clinical manifestations can occur at any age – from the neonatal period to old age. Despite the well-studied etiopathogenetic and clinical-diagnostic aspects of the disease, the issues of diagnosis and treatment tactics for Minkowski-Chauffard anemia in the neonatal period raise a number of controversial issues. Features of fetal hematopoiesis (physiological macrocytosis in newborns, a large amount of fetal hemoglobin, physiological hemolysis, a significant number of immature erythrocyte forms) complicate the early diagnosis of the disease. The article deals data on hemolytic anemia – MinkowskiShoffar disease: causes, pathogenesis, clinical signs, severity, features of diagnosis and treatment This article discusses two cases of hereditary microspherocytosis that manifested in the neonatal period; The regenerative functions of hematopoiesis were analyzed (reticulocyte level in absolute numbers and in %, reticular index, RET-He, Delta-He, IRF, LFR, MFR, HFR) in specific patients.

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