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The authors described the patient as presenting with a haemolytic crisis but for more than 50 years recurrent episodes of anaemia in a patient with Hereditary Spherocytosis have been known to be aregenerative and not haemolytic crises. What happens is that the patient has some intercurrent infection. Bone marrow function is depressed, marrow production of red cells is depressed, haemolysis continues and the haemoglobin level falls. When the effect of the infection settles, erythropoiesis improves, there is a prominent reticulocytosis and the haemoglobin level rises.
An unusual feature in the patient described was that ultrasound examination of the abdomen showed splenomegaly with no mention of gallstones. In an adult with Hereditary Spherocytosis, often the gallbladder is filled withe small black pigment stones and then splenectomy needs to be accompanied by cholecystectomy once the various immunizations have been carried out.
One does meet occasional patients with Hereditary Spherocytosis in whom the disorder seems to be a spontaneous mutation but mostly it is inherited as an autosomal dominant disorder with Hereditary Spheroctosis in one parent and with a 50/50 chance of being present in siblings and children.