In this report a sixth case of a storage disorder of unknown etiology is described and other reported cases are discussed. No hereditary or significant etiologic factors were noted in these patients. The disease is characterized by a benign course, splenomegaly and in some cases thrombocytopenia. A lipid containing histiocyte with unique blue staining granular cytoplasm is present in bone marrow and splenic smears, and biochemically the storage material consists largely of sphingomyelin.

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