Tunç B, Ayata A
Department of Pediatric Hematology, Süleyman Demirel University, Isparta, Turkey.
Pediatr Hematol Oncol. 2001 Dec;18(8):531-6. doi: 10.1080/088800101753328501.
The authors report a case of hemophagocytic syndrome (HPS) associated with acute visceral leishmaniasis (VL). A 4-year-old boy was admitted with high fever, hepatosplenomegaly, and pancytopenia. Elevated serum ferritin and triglyceride, low fibrinogen levels, and bone-marrow (BM) histiocytic hyperplasia with prominent hemophagocytosis were consistent with a HPS. An initial diagnosis of kala-azar was refuted because of negativity of BM aspiration and serology for this parasite, and the diagnosis HPS was made. Three months after first admission, reevaluation of the BM aspiration revealed many amastigotes of Leishmania parasites. The serology of VL became positive, finally establishing the diagnosis of VL. Although specific therapy for VL was instituted, the patient died 4 weeks after the diagnosis.
作者报告了一例与急性内脏利什曼病(VL)相关的噬血细胞综合征(HPS)。一名4岁男孩因高热、肝脾肿大和全血细胞减少入院。血清铁蛋白和甘油三酯升高、纤维蛋白原水平降低以及骨髓(BM)组织细胞增生伴明显噬血细胞现象与HPS相符。由于骨髓穿刺和该寄生虫血清学检查均为阴性,最初排除了黑热病的诊断,诊断为HPS。首次入院三个月后,再次进行骨髓穿刺复查发现了许多利什曼原虫无鞭毛体。VL血清学检查转为阳性,最终确诊为VL。尽管对VL进行了特异性治疗,但患者在诊断后4周死亡。