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How is hereditary spherocytosis (Minkowski-Schoffar's disease) treated?
Medical expert of the article
Last reviewed: 19.10.2021
Treatment
Assign folic acid to 1 mg per day during the hemolysis.
In case of severe hemolytic or aplastic crisis (hemoglobin <60 g / l), substitution therapy with erythrocyte mass, purified from leukocytes, is carried out.
Splenectomy is used in a planned manner. Before the operative treatment it is necessary to vaccinate a patient against meningococcal and pneumococcal infections, as well as hemophilic infection of type B.
In cholelithiasis, cholecystectomy is used.
Dispensary supervision
Recommend an examination of the hematologist, a general blood test with the calculation of reticulocytes (once a month with moderate and severe forms, once a quarter - with light and minimal forms).
The biochemical analysis of blood (determination of the content of total and indirect bilirubin, activity of ALT, ACT, alkaline phosphatase, LDH, concentration of SJ, OZHSS, saturation of transferrin with iron, SF) is carried out once a quarter with moderate and severe forms, every 6 months - with mild and minimal forms.
Ultrasound of the abdominal cavity is recommended every 6-12 months.