Rajagopala Srinivas, Dutta Usha, Chandra K S Poorna, Bhatia Prateek, Varma Neelam, Kochhar Rakesh
Department of Pulmonary & Critical Care Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India.
J Infect. 2008 May;56(5):381-8. doi: 10.1016/j.jinf.2008.02.013. Epub 2008 Apr 10.
The clinical features of leishmaniasis overlap with that of hemophagocytic lymphohistiocytosis (HLH) and the diagnosis of visceral leishmaniasis (VL) related HLH can be challenging.
To review information available on disease course, treatment, adjunctive therapy used and the outcomes of VL related HLH.
We describe an illustrative case and review all reported cases of VL associated HLH in the English literature till March 2007.
VL associated HLH is rare, with 56 cases reported in the English literature. Clinical features lack discriminating value to recognize VL as the inciting etiology. Bone marrow aspiration (BMA) establishes the diagnosis in 78% of cases but is often negative at onset of the syndrome due to the pauci-microbial nature of the disease and patchy involvement. Repeated marrow aspiration, liver biopsy, blood cultures and serology may be required to establish the diagnosis. Liposomal amphotericin is the drug of choice. IVIG may be considered when there is an inadequate response to anti-leishmanial therapy in severe and refractory disease.
VL related HLH is often under-recognized because of overlapping clinical features and negative marrow evaluation at onset, leading to high mortality rates.
利什曼病的临床特征与噬血细胞性淋巴组织细胞增生症（HLH）重叠，内脏利什曼病（VL）相关HLH的诊断具有挑战性。
综述关于VL相关HLH的病程、治疗、辅助治疗及预后的现有信息。
我们描述一个典型病例，并回顾截至2007年3月英文文献中所有报道的VL相关HLH病例。
VL相关HLH较为罕见，英文文献中共报道了56例。临床特征对于识别VL作为致病病因缺乏鉴别价值。骨髓穿刺（BMA）在78%的病例中可确诊，但由于疾病微生物数量少且呈局灶性累及，在综合征发作时往往为阴性。可能需要多次骨髓穿刺、肝活检、血培养和血清学检查来确诊。脂质体两性霉素是首选药物。在严重和难治性疾病中，抗利什曼治疗反应不佳时可考虑静脉注射免疫球蛋白（IVIG）。
由于临床特征重叠以及发作时骨髓评估为阴性，VL相关HLH常未得到充分认识，导致高死亡率。
