Rajagopala Srinivas, Singh Navneet
Department of Pulmonary, Medicine and Liver Intensive Care, Global Hospitals and Health City Cheran Nagar, Perumbakkam, Chennai, India.
Acta Med Acad. 2012;41(2):161-74. doi: 10.5644/ama2006-124.49.
Hemophagocytic lymphohistiocytosis (HLH) is a catastrophic syndrome of unrestrained immune activation. Evaluation and management of HLH in the tropics is challenging.
To examine the reported etiologies and management of HLH reported from the sub-continent.
Systematic review of all published cases from the Indian sub-continent.
We found only 156 published cases of HLH from the sub-continent. HLH was reported from the immediate perinatal period to 46 years of age. Infection-associated HLH (IAHS) constituted 46.8% of all cases of HLH (44% and 51% in children and adults respectively). In adults, tropical infections triggered 51% of these cases of IAHS. Steroids were used in 47% of children and 10% of adults. Etoposide and/or cyclosporine were used in 8% children and 8% of adults only. Intravenous immunoglobulin was used in another 30% of children and 4% of the adults. HLH-related mortality occurred in 31.8% and 28% of children and adults respectively.
HLH is under-reported in the sub-continent and has high mortality. Cyclosporine and etoposide are seldom administered early despite diagnosis of HLH. Larger cohorts with IAHS triggered by tropical infections are urgently needed to understand its natural history and implications of this differing prescription pattern on mortality.
噬血细胞性淋巴组织细胞增生症（HLH）是一种免疫激活不受控制的灾难性综合征。在热带地区对HLH进行评估和管理具有挑战性。
研究次大陆报道的HLH的病因及管理情况。
对印度次大陆所有已发表病例进行系统综述。
我们仅发现次大陆有156例已发表的HLH病例。HLH的报道年龄范围从围生期即刻到46岁。感染相关HLH（IAHS）占所有HLH病例的46.8%（儿童和成人分别为44%和51%）。在成人中，热带感染引发了这些IAHS病例的51%。47%的儿童和10%的成人使用了类固醇。仅8%的儿童和8%的成人使用了依托泊苷和/或环孢素。另外30%的儿童和4%的成人使用了静脉注射免疫球蛋白。HLH相关死亡率在儿童和成人中分别为31.8%和28%。
HLH在次大陆的报道不足且死亡率高。尽管已诊断出HLH，但环孢素和依托泊苷很少早期使用。迫切需要更大规模的由热带感染引发的IAHS队列，以了解其自然病史以及这种不同处方模式对死亡率的影响。
