Kanchi Kamakoti CHILDS Trust Hospital, Nungambakkam, Chennai 600 034, India.
Indian Pediatr. 2011 Jan;48(1):31-5. doi: 10.1007/s13312-011-0020-2. Epub 2010 Aug 1.
To describe the epidemiology, clinical features, laboratory findings, outcome and the difficulties in diagnosis and management of children with Hemo-phagocytic Lymphohistiocytosis (HLH) in a tertiary children's hospital in India.
Retrospective analysis of case records of all the children with a diagnosis of HLH from December 2006 to December 2008.
Tertiary care children's teaching hospital in Chennai, India.
43 children had a diagnosis of hemo-phagocytosis, of who only 33 (19 male, mean age 46 months, range 50 days-14 years) met the inclusion criteria based on the HLH 2004 protocol of the Histiocyte Society. The predominant presenting features included prolonged fever and hepatosplenomegaly. CNS symptoms were present in 36%. Anemia (Hb <9 gm/dL), and thrombocytopenia (platelets <1,00,000/mm3) were present in 97% and 72%, respectively. Among the biochemical markers, hyperferritinemia was present in 97%, and hypofibrinogenemia and high LDH in 92%. Bone marrow examination showed hemophagocytosis in 84%. Infectious agents were identified in 42% children, with viruses accounting for 2/3 of them (5 Dengue virus, 3 EBV, 1 CMV, 1 TB and 5 bacterial agents). The mean duration between the onset of symptoms and the diagnosis was 16 days. Corticosteroids were the most commonly used immunomodulatory agents (67%), followed by IVI g (64%). Cyclosporine was used in 33% and Etoposide in 15%. Improvement of laboratory parameters was noticed within 5-7 days of starting treatment. Overall survival rate was 76%.
HLH should be considered in the differential diagnosis of children with prolonged fever, hepatosplenomegaly and cytopenia. Prompt recognition and appropriate therapy may result in good outcome, particularly in Infection associated HLH.
描述印度一家三级儿童医院儿童噬血细胞性淋巴组织细胞增生症（HLH）的流行病学、临床特征、实验室检查结果、转归以及诊断和治疗的难点。
对 2006 年 12 月至 2008 年 12 月期间所有被诊断为 HLH 的患儿的病历进行回顾性分析。
印度钦奈的一家三级儿童教学医院。
共有 43 名患儿被诊断为噬血细胞，其中仅 33 名（19 名男性，平均年龄 46 个月，范围 50 天至 14 岁）符合 2004 年组织细胞协会 HLH 方案的纳入标准。主要表现为发热持续时间长和肝脾肿大。中枢神经系统症状占 36%。贫血（Hb<9gm/dL）和血小板减少症（血小板<1,00,000/mm3）分别占 97%和 72%。在生化标志物中，高血清铁蛋白血症占 97%，低纤维蛋白原血症和高乳酸脱氢酶血症占 92%。骨髓检查显示 84%有噬血细胞现象。42%的患儿确定了感染病原体，其中病毒占 2/3（5 例登革热病毒、3 例 EBV 病毒、1 例 CMV 病毒、1 例结核分枝杆菌和 5 例细菌）。从症状出现到诊断的平均时间为 16 天。最常用的免疫调节药物是皮质类固醇（67%），其次是 IVI g（64%）。环孢素在 33%的患儿中使用，依托泊苷在 15%的患儿中使用。开始治疗后 5-7 天内，实验室参数改善。总生存率为 76%。
对于发热时间长、肝脾肿大和血细胞减少的患儿，应考虑 HLH 的鉴别诊断。及时识别和适当治疗可能会取得良好的转归，特别是感染相关性 HLH。
