Systemic giant-cell histiocytosis: report of a case and a review of the adult form of Letterer-Siwe disease.
The multiple classifications of histiocytic disorders, and the lack of specificity in their application, have degraded the utility of such diagnostic terms as "Letterer-Siwe Disease" and "Malignant Histiocytosis". A case is presented of a systemic histiocytic proliferation with erythrophagocytosis and giant-cell formation which does not fit into any presently accepted diagnostic niche. No similar case was found in the literature. Moreover, it appears that the entity "Adult form of Letterer-Siwe disease" has been uncritically accepted on the basis of a few poorly documented cases, and should be abandoned.
D000328:Adult; D000368:Aged; D001853:Bone Marrow / Q000473:pathology; D003937:Diagnosis, Differential; D006529:Hepatomegaly / Q000473:pathology; D006644:Histiocytes / Q000473:pathology; D006646:Histiocytosis, Langerhans-Cell / Q000473:pathology*; D006801:Humans; D007031:Hypothalamus / Q000473:pathology; D008206:Lymphatic Diseases / Q000473:pathology; D008297:Male; D010179:Pancreas / Q000473:pathology; D010902:Pituitary Gland / Q000473:pathology; D012867:Skin / Q000473:pathology; D013163:Splenomegaly / Q000473:pathology