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The authors report five cases of familial erythrophagocytic lymphohistiocytosis (F.E.L.H.). Two patients are brothers. One case is associated with a striking extramedullary hematopoiesis. A cytophotometric study of nuclear DNA in the histiocytes was performed in all cases. The histiocytes are diploid and there is no aneuploidy. These findings allow the authors to exclude the hypothesis of the tumoral nature of F.E.L.H. The data collected from the literature about the familial incidence of F.E.L.H. militate for an autosomal recessive transmission. The extramedullary hematopoiesis, which has not yet been reported in association with F.E.L.H., is probably due to the intense lymphohistiocytic infiltration of the bone marrow.
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