Sinus histiocytosis with massive lymphadenopathy. An analysis of 14 deaths occurring in a patient registry

摘要: Sinus histiocytosis with massive lymphadenopathy (SHML) is generally regarded as a benign disorder in spite of its propensity to form large masses and disseminate both nodal extranodal sites. Although most patients the extent SHML does not appear determine disease outcome, recent reports have documented that infiltrates can cause death. To gain perspective on role patient deaths this population, authors analyzed 14 known fatalities an registry comprising 215 patients. The average age at death was 33 years. Autopsy performed eight were clearly only two (previously reported). In another four cases persistent prominent other result complications defined immunologic abnormalities (five), or unusual infections (three). seen example cytologic malignant transformation SHML.