Bourquelot P, Oksenhendler E, Wolff M, Fegueux S, Piketty C, D'Agay M F, Clauvel J P
Service d'Immuno-Hématologie, Hôpital Saint-Louis, Paris.
Presse Med. 1993 Sep 11;22(26):1217-20.
This report describes the clinical spectrum and outcome of the hemophagocytic syndrome (HS) in 5 HIV infected patients. All 5 patients presented with fever, hepatomegaly and/or splenomegaly, confusion or coma and respiratory symptoms. Severe anemia was associated with thrombocytopenia and with neutropenia in 4 cases. Diffuse intravascular coagulopathy was present in 2 cases. Liver function tests were abnormal in three patients. The diagnosis of HS was made 2 to 12 weeks after the onset of symptoms and required in most patients repeated examinations of the bone-marrow, showing infiltration by histiocytes with prominent phagocytosis of blood cells. In one case this infiltration was not seen in the bone-marrow but only in the liver and the spleen. Varicella, mycobacterium infection, oesophageal candidiasis, Kaposi sarcoma were observed in the evolution of 3 patients. Anaplastic large cell Ki-1 lymphoma was present in one case. Four patients died as a result of complications of HS. The one patient with lymphoma survived.
本报告描述了5例HIV感染患者噬血细胞综合征（HS）的临床谱及转归。所有5例患者均有发热、肝肿大和/或脾肿大、意识模糊或昏迷以及呼吸道症状。4例患者出现严重贫血，伴有血小板减少和中性粒细胞减少。2例出现弥漫性血管内凝血。3例患者肝功能检查异常。HS的诊断在症状出现后2至12周作出，大多数患者需要反复进行骨髓检查，显示组织细胞浸润并伴有明显的血细胞吞噬现象。1例患者骨髓中未见这种浸润，仅在肝脏和脾脏中可见。3例患者病程中出现水痘、分枝杆菌感染、食管念珠菌病、卡波西肉瘤。1例患者患有间变性大细胞Ki-1淋巴瘤。4例患者死于HS并发症。1例淋巴瘤患者存活。
