Mishra Sataroopa, Singh Amitabh, Singh Lavleen, Mandal Anirban, Jain Rahul
Department of Pediatrics, Chacha Nehru Bal Chikitsalaya, New Delhi, India.
Department of Pathology, Chacha Nehru Bal Chikitsalaya, New Delhi, India.
Asian J Transfus Sci. 2018 Jan-Jun;12(1):85-88. doi: 10.4103/ajts.AJTS_142_16.
Hemophagocytic lymphohistiocytosis (HLH) is a disorder of immune dysregulation secondary to a massive unregulated cytokine storm and its downstream consequences. HLH is being increasingly recognized as a cause of pyrexia of unknown origin, unexplained cytopenias, and hepatic dysfunction. However, this potentially treatable condition is often missed due to lack of suspicion, variable, and nonspecific presentations, inability to fulfil all the diagnostic criteria and availability of diagnostic tests in resource limited settings. Both familial and acquired forms of HLH can be triggered by multiple factors in a susceptible patient. We report a 9-month old infant who developed HLH in association with Stevens-Johnson syndrome following massive blood transfusion.
噬血细胞性淋巴组织细胞增生症（HLH）是一种继发于大规模不受控制的细胞因子风暴及其下游后果的免疫失调疾病。HLH越来越被认为是不明原因发热、不明原因血细胞减少和肝功能障碍的一个病因。然而，由于缺乏怀疑、表现多样且不具特异性、无法满足所有诊断标准以及资源有限环境中诊断测试的可及性，这种潜在可治疗的病症常常被漏诊。HLH的家族性和获得性形式在易感患者中均可由多种因素触发。我们报告一名9个月大的婴儿，在大量输血后并发史蒂文斯-约翰逊综合征而发生HLH。
