抄録
A twenty-one-year-old male subject had been complaining of anemic symptoms and bleeding tendency of several years duration, and was incidentally found to have mild hepatomegaly at the age of nineteen. The clinical picture of aplastic anemia was associated with foam cells infiltrating into the bone marrow and lymph nodes, and peripheral leukocytes having vacuolization in the cytoplasm. A slight degree of mental retardation was noted, but otherwise normal neurologically. The characteristic foam histiocytes were filled with many PAS-negative granules which fluoresced strongly a greenish-yellow in ultraviolet light. However, biochemical analysis of lipids accumulated in the cytoplasm of foam cells remains to be conclusive. Total sphingomyelinase activity was slightly below that of normal control. These findings were compatible in every way with those described with adult Niemann-Pick disease. The relationship of foam cells to various lipid storage diseases, both inherited and acquired, was discussed.
