The liver was 2 cm below the right costal margin and the spleen was 3 cm below the left costal margin. The lymph nodes were normal. Coombs tests were negative. The blood smear revealed that most of her red blood cells were spherocytic in shape. Hereditary spherocytosis is an autosomal dominant disease characterized by a defect in the red blood cell cytoskeleton .
Hemolytic crisis is the most common presentation of hereditary spherocytosis, as shown above.
Excessive hemolysis can be seen as early as the first 24 hours of life, causing early and prolonged neonatal jaundice . Hypoplastic crisis , due to viral-induced bone marrow suppression, is another common manifestation of this disease. Parvovirus-like viruses are particularly common etiologies. Treatment with blood transfusion may be necessary in cases of acute hemolysis or prolonged hypoplastic crises. Osmotic fragility test measures the increased sensitivity of red cells to hypotonic saline.