FAMILIAL RETICULOENDOTHELIOSIS: CONCURRENCE OF DISEASE IN FIVE SIBLINGS

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Abstract
A fatal syndrome characterized by failure to thrive, eczema, recurrent skin, ear, and respiratory tract infections, lymphadenopathy hepatosplenomegaly, pulmonary infiltrations, and terminal peripheral pancytopenia and hypergammaglobulinemia is described in a complete sibship of five girls. Histologically, there was diffuse reticulum cell infiltration in the lungs, liver, spleen, lymph nodes, bone marrow, gastrointestinal and genitourinary tracts, and central nervous system. The lymphoid architecture was obliterated and there was a marked plasmacytosis. Death occurred between the ages of 20 months and 4 9/12 years and was associated with pulmonary insufficiency, septicemia, and candidiasis. Attempts to identify an etiologic agent were unsuccessful. Although similar to other reviewed cases of familial reticuloendotheliosis, the involvement of a complete sibship appears to be a unique occurrence.

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