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How is hereditary spherocytosis (Minkowski-Schoffar disease) treated?
Last reviewed: 06.07.2025
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Folic acid is prescribed at 1 mg per day during the period of hemolysis.
In severe hemolytic or aplastic crisis (hemoglobin <60 g/l), replacement therapy is performed with red blood cell mass purified from leukocytes.
Splenectomy is used on a planned basis. Before surgical treatment, it is necessary to vaccinate the patient against meningococcal and pneumococcal infections, as well as Haemophilus influenzae type B.
In case of gallstone disease, cholecystectomy is used.
Outpatient observation
They recommend an examination by a hematologist, a general blood test with a reticulocyte count (once a month for moderate and severe forms, once a quarter for mild and minimal forms).
A biochemical blood test (determination of total and indirect bilirubin, ALT, AST, alkaline phosphatase, LDH activity, SF, TIBC concentration, transferrin saturation with iron, SF) is performed once a quarter for moderate and severe forms, once every 6 months for mild and minimal forms.
An ultrasound of the abdominal organs is recommended once every 6-12 months.