Devi Kanta, Ali Natasha
Department of Oncology, Aga Khan University, Karachi, Pakistan.
Department of Pathology and Laboratory Medicine/Oncology, Aga Khan University, Karachi, Pakistan.
IDCases. 2021 Aug 31;26:e01275. doi: 10.1016/j.idcr.2021.e01275. eCollection 2021.
HLH is a progressive syndrome of unchecked immune activation and tissue damage. If left untreated, patients with HLH survive for only a few months, due to progressive multi-organ failure. Prompt initiation of treatment for HLH is essential for the survival of affected patients. Several conditions are responsible for triggering HLH in clinically stable patients who respond to treatment of the underlying condition alone. These conditions include infection, rheumatological diseases and lymphoid malignancies. We report a rare case of primary HLH in a 32-year-old female who presented with fever, abdominal pain, pancytopenia and splenomegaly with the triggering factor being Dengue infection.
噬血细胞性淋巴组织细胞增生症（HLH）是一种免疫激活失控和组织损伤进行性发展的综合征。若不治疗，HLH患者由于进行性多器官功能衰竭，生存期仅为数月。及时开始HLH治疗对受影响患者的生存至关重要。在仅对基础疾病进行治疗即有反应的临床稳定患者中，有几种情况可引发HLH。这些情况包括感染、风湿性疾病和淋巴系统恶性肿瘤。我们报告一例罕见的原发性HLH病例，患者为一名32岁女性，表现为发热、腹痛、全血细胞减少和脾肿大，触发因素为登革热感染。
