Malignancy associated hemophagocytic lymphohistiocytosis in children
Malignancy associated hemophagocytic lymphohistiocytosis in children
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DOI：
10.4103/0973-1482.188437
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发表时间：
2018-04-01
影响因子：
1.3
通讯作者：
Seth, Rachna
中科院分区：
文献类型：
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作者：
Singh, Amitabh;Dawman, Lesa;Seth, Rachna
研究目的：噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的免疫失调性疾病，导致异常的T细胞活化和炎性细胞因子产生，产生一系列HLH特有的临床特征。小儿继发性HLH通常由感染、恶性肿瘤或风湿性疾病引发。恶性肿瘤相关HLH（MA-HLH）的诊断是一个困难的挑战，因为临床特征可能归因于基础疾病或化疗。我们的研究目的是看到这种罕见的实体在我们centre.Materials和方法的发生：数据收集从所有儿科肿瘤患者在我们的中心治疗的诊断MA-HLH从2012年1月至2014年12月。收集数据的年龄，性别，基础疾病，治疗方案，化疗阶段，任何潜在的感染，治疗给予HLH，outcome.Results：有5例患者的诊断MA-HLH在研究期间。年龄范围为18个月至9岁。在5例MA-HLH中，2例患者患有急性淋巴细胞白血病，2例急性髓细胞白血病，1例患有霍奇金淋巴瘤。三名记录有微生物感染的患者在适当治疗后也没有改善。两名患者在治疗期间死亡。1例患者在单独使用类固醇后完全改善。结论：儿童MA-HLH的诊断延迟可能是由于对病情认识不足，及时诊断是提高疗效的关键。在此，我们讨论我们的经验，这种罕见的实体在儿科肿瘤患者的文献回顾。
Aim of Study: Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder of immune dysregulation resulting in abnormal T-cell activation and inflammatory cytokine production which produces a constellation of clinical features unique to HLH. Pediatric secondary HLH is usually triggered by infection, malignancy, or rheumatological disorders. The diagnosis of malignancy-associated HLH (MA-HLH) poses a difficult challenge as clinical features may be attributed to the underlying disease or chemotherapy. Our study aimed to see the occurrence of this rare entity at our centre.Materials and Methods: Data were collected from all pediatric oncology patient treated at our center with the diagnosis of MA-HLH from January 2012 to December 2014. Data were collected for age, sex, underlying disease, treatment protocol, stage of chemotherapy, any underlying infection, treatment given for HLH, and outcome.Results: There were five patients with a diagnosis of MA-HLH in the study period. Age ranged from 18 months to 9 years. Of the five MA-HLH, two patients had acute lymphoblastic leukemia, two acute myeloid leukemia, and one had Hodgkin lymphoma. The three patients who had documented microbiological infection also did not improve after appropriate treatment. Two patients died during treatment. One patient improved completely on steroid alone. One patient received HLH 2004 induction.Conclusion: The delay in the diagnosis of MA-HLH in pediatric patients may be due to decrease awareness about the condition the timely diagnosis of MA-HLH is crucial for a better outcome. Herein, we discuss our experience with this rare entity in pediatric oncology patients with review of literature.