Ozyürek Emel, Ozçay Figen, Yilmaz Basak, Ozbek Namik
Department of Pediatrics, Başkent University, Faculty of Medicine, Adana Teaching and Medical Research Center, Adana, Turkey.
Pediatr Hematol Oncol. 2005 Jul-Aug;22(5):409-14. doi: 10.1080/08880010590964309.
Leishmania-associated hemophagocytic lymphohistiocytosis is a rare clinicopathological entity. This condition is often difficult to diagnose, so treatment is often delayed. This report describes the case of a 5-year-old boy who was admitted with fever of 1 month's duration, hepatosplenomegaly, and pancytopenia. Serum testing showed elevated transaminase levels, hypertriglyceridemia, hyperferritinemia, and normal fibrinogen level. Hemophagocytic lymphohistiocytosis was diagnosed on bone marrow examination. The patient was tested for various infectious agents. He was negative for all except Leishmania, which was detected by indirect fluorescent antibody testing. Treatment with amphotericin B resulted in a dramatic resolution of all signs and symptoms within 1 week.
利什曼原虫相关噬血细胞性淋巴组织细胞增生症是一种罕见的临床病理实体。这种病症常常难以诊断，因此治疗往往被延误。本报告描述了一名5岁男孩的病例，该男孩因持续1个月的发热、肝脾肿大和全血细胞减少入院。血清检测显示转氨酶水平升高、高甘油三酯血症、高铁蛋白血症以及纤维蛋白原水平正常。骨髓检查确诊为噬血细胞性淋巴组织细胞增生症。对该患者进行了多种感染因子检测。除利什曼原虫外，其他检测均为阴性，利什曼原虫通过间接荧光抗体检测被发现。使用两性霉素B治疗后，所有体征和症状在1周内显著缓解。
