Hereditary xerocytosis revisited.

摘要: A 21 year old male student presented in 1980 as an Olympic athlete with a 12 history of jaundice, pallor, and darkened urine induced by the atraumatic exercise swimming (1). Physical examination at that time was remarkable only for moderate scleral icterus without hepatosplenomegaly. Hematological revealed macrocytosis (MCV 102 fL) anemia (Hct 50%, Hb 17 g/dL, 9% reticulocytes). The peripheral blood smear showed occasional target cells. Red cell osmotic fragility decreased. Na content increased K decreased, reduced total monovalent ion content. Passive red permeability both were increased. supervised 2.5 hr workout free plasma from <5 to 45 mg/dL decreased serum haptoglobin 25 6 mg/dL. post-exercise sediment hemosiderin-laden tubular epithelial cells, frank hemoglobinuria. circulating 15 day erythrocyte half-life measured after days further shortened resumption twice-per-day workouts 1 week. patient’s cells hypersensitive vitro shear stress applied cone-plate viscometer.