oa South African Medical Journal - Mediterranean anaemia : an investigation of nineteen cases in a single family



Nineteen cases of Mediterranean anaemia in a single family are descriptionbed. On clinical, haematological and radiological grounds they fall clearly into three groups: Group I: Cases showing well-marked hypochromia, icterus, spenomegaly, normoblasts in the peripheral blood, and an elevated reticulocyte count. The bone marrow showed marked normoblastic hyperplasia, and radiological changes were present. The females in this group showed chronic ulceration over the malleoli. Group II: Cases showing hypochromia, either low or normal haemoglobin values, but usually high red cell counts (6-7 million cells per c.mm.), and no icterus. The bone marrow may show a moderate degree of normoblastic hyperplasia. Group III: Cases showing evidence of the trait, viz. the red cells have an increased resistance to saline haemolysis. Target cells are present in the peripheral blood. The pathogenesis of the condition is discussed.


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