Splenic histiocytosis in idiopathic thrombocytopenic purpura: a relative sphingomyelinase deficiency?
The case of a patient in whom idiopathic thrombocytopenic purpura (ITP) was associated with diffuse splenic histiocytosis is described; the patient's subsequent sphingomyelinase level was at the lower limits of the normal range. The patient's splenic lecithin:sphingomyelin ratio was not significantly different from that of 11 age-matched control subjects. It is postulated that the sporadic cases of splenic histiocytosis in patients with ITP are due to a relative, acquired sphingomyelinase deficiency.
D000293:Adolescent; D006801:Humans; D008206:Lymphatic Diseases / Q000209:etiology / Q000473:pathology*; D008297:Male; D010727:Phosphoric Diester Hydrolases / Q000172:deficiency*; D011696:Purpura, Thrombocytopenic / Q000473:pathology*; D013108:Sphingomyelin Phosphodiesterase / Q000172:deficiency*; D013158:Splenic Diseases / Q000209:etiology / Q000473:pathology*
D010727:Phosphoric Diester Hydrolases; D013108:Sphingomyelin Phosphodiesterase