Splenic pseudosinuses and hepatic angiomatous lesions. Distinctive features of hairy cell leukemia.
In 14 cases of hairy cell leukemia unique vascular lesions not previously reported were observed in surgically resected spleens and hepatic biopsies. In all 14 spleens there were variably prominent distended spaces filled with erythrocytes and resembling dilated sinuses. These structures appeared to be lined by hairy cells,and studies utilizing histochemical and enzymatic methods confirmed that they were in fact pseudosinuses lacking the endothelial cells and ring fibers of normal splenic sinuses. These changes were not present in normal spleens or in spleens involved by other types of chronic leukemia, and appear to be qualitatively specific for hairy cell leukemia. In three of five hepatic biopsies similar angiomatous lesions were present in multifocal clusters resembling hemangiomas. Their pathogenesis may be related to possible adherence of hairy cells to reticulum fibers in involved tissues.
D001706:Biopsy; D004727:Endothelium / Q000166:cytology / Q000201:enzymology; D006391:Hemangioma / Q000473:pathology; D006651:Histocytochemistry; D006801:Humans; D007938:Leukemia / Q000473:pathology*; D007945:Leukemia, Lymphoid / Q000473:pathology; D007951:Leukemia, Myeloid / Q000473:pathology; D008113:Liver Neoplasms / Q000473:pathology*; D008206:Lymphatic Diseases / Q000473:pathology*; D009024:Morphogenesis; D009283:Naphthol AS D Esterase / Q000378:metabolism; D013158:Splenic Diseases / Q000473:pathology; D013162:Splenic Vein / Q000473:pathology*; D014652:Vascular Diseases / Q000473:pathology
D009283:Naphthol AS D Esterase