FAMILIAL ERYTHROPHAGOCYTIC LYMPHOHISTIOCYTOSIS

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Abstract
The clinical records and gross and histological findings are described in the case of two siblings who died at different times and at slightly different ages of an unusual, but apparently similar, disease complex. Letterer-Siwe's disease was suspected clinically, but the autopsy findings characterized by hyperplasia of histiocytic cells, erythrophagocytosis, and an infiltration of lymphocytes and histiocytes into almost every organ in the body, including, in one of the children, the central nervous system, were distinctly different from the disease described by Letterer. Neither the etiology nor the nature of the disease is understood. Little or nothing is known about therapy though splenectomy in one of the two infants led to a temporary relief. Its similarity to other familial diseases of infancy designated by such terms as familial haemophagocytic reticulosis, familial nonlipid reticuloendotheliosis, familial generalized lympho-histiocytic infiltration, and possibly, Chédiak-Higashi syndrome, is so striking as to suggest that these exceptional diseases may themselves comprise a distinct clinical and anatomical category. Until more is known about this group, the descriptive term "familial erythrophagocytic lympho-histiocytosis" is suggested.

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