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May 1958

Hereditary Spherocytosis: Is the Duration of the "Aplastic" Crisis Influenced by Splenectomy?

Author Affiliations

U. S. A. F.; Oklahoma City

From the Departments of Medicine and Surgery, The University of Oklahoma School of Medicine and University Hospitals.

AMA Arch Intern Med. 1958;101(5):894-898. doi:10.1001/archinte.1958.00260170050007

There is general agreement that hereditary spherocytosis is characterized by a chronic and continuous hemolytic process which results from an inborn error in the glycolytic metabolism of the spherocytes.1,2 Whether anemia exists or not has been thought to reflect the ability of erythropoiesis to compensate for this increased and random destruction of red cells. The pathogenesis of the acute crisis, however, has been variously formulated. One generally held concept of the crisis visualizes a sudden increase in the rate of hemolysis.3 The factors which might be responsible for this are still debated. In 1948, Owren reported the details regarding six cases of acute crisis in which the findings of anemia, thrombocytopenia, and reticulocytopenia were associated with hypoplasia of the erythroid elements in the bone marrow.4 Owren questioned the concept of increased hemolysis and postulated that an "aplastic crisis" of the marrow accounted for the sudden fall in

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