Obayo Antonina, Sharma Karishma, Mithi Caroline, Riyat Malkit, Mwirigi Anne
Department of Medicine Faculty of Health Sciences Aga Khan University Medical College of East Africa Nairobi Kenya.
Department of Haematology and Oncology, Faculty of Health Sciences Aga Khan University Medical College of East Africa Nairobi Kenya.
EJHaem. 2020 Oct 13;1(2):567-575. doi: 10.1002/jha2.113. eCollection 2020 Nov.
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive inflammation and tissue destruction due to abnormal immune activation. HLH carries a very high mortality, and while delays in patients' presentation to hospital, time to suspicion of HLH, investigation, and initiation of therapy all play a part, mortality remains high even with timely diagnosis and treatment. Classical manifestations of HLH include persistent fever, cytopenias, and liver dysfunction.
We present four cases of secondary HLH, highlighting the demographic and clinical characteristics of these patients, underlying triggers (including systemic lupus erythematosus, lymphoproliferative disorders, and leishmaniasis), together with challenges associated with the diagnosis and treatment of this rare disorder and a brief review of literature.
HLH has protean manifestations and requires a high index of suspicion as it can be a great clinical masquerader. Mortality due to multiorgan failure is often high even with early recognition and treatment.
噬血细胞性淋巴组织细胞增生症（HLH）是一种由于异常免疫激活导致过度炎症反应和组织破坏的综合征。HLH的死亡率非常高，尽管患者就诊延迟、怀疑HLH的时间、检查以及开始治疗等因素都有影响，但即使及时诊断和治疗，死亡率仍然很高。HLH的典型表现包括持续发热、血细胞减少和肝功能障碍。
我们报告了4例继发性HLH病例，重点介绍了这些患者的人口统计学和临床特征、潜在诱因（包括系统性红斑狼疮、淋巴增殖性疾病和利什曼病），以及与这种罕见疾病的诊断和治疗相关的挑战，并对文献进行了简要综述。
HLH有多种表现形式，由于它可能是一个严重的临床伪装者，因此需要高度怀疑。即使早期识别和治疗，多器官功能衰竭导致的死亡率通常也很高。
