Lymphadenopathy as the initial manifestation of histiocytosis X.
Seventeen cases of histiocytosis X presenting as lymphadenopathy, in which the initial diagnosis was based on lymph node biopsy, are reviewed, the characteristic histopathologic findings described, and the differential diagnosis discussed. Clinical evaluation reveals a broad spectrum of associated manifestations varying from solitary eosinophilic granuloma of lymph node to a disseminated Letterer-Siwe-like syndrome. Follow-up confirms the essentially benign nature of this disorder when defined by strict histologic criteria. The implications of these findings on the continuing controversy surrounding histiocytosis X are discussed.
D000293:Adolescent; D000328:Adult; D001847:Bone Diseases / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006644:Histiocytes / Q000648:ultrastructure; D006646:Histiocytosis, Langerhans-Cell / Q000473:pathology*; D006801:Humans; D007223:Infant; D007801:Langerhans Cells / Q000473:pathology; D008198:Lymph Nodes / Q000473:pathology; D008206:Lymphatic Diseases / Q000473:pathology* / Q000628:therapy; D008297:Male