成人噬血细胞性淋巴组织细胞增生症：一种未被充分认识的疾病。
Hemophagocytic lymphohistiocytosis in adults: An under recognized entity.
作者信息
Shah Abdul Rashid, Muzzafar Tariq, Assi Rita, Schellingerhout Dawid, Estrov Zeev, Tamamyan Gevorg, Kantarjian Hagop, Daver Naval
机构信息
Department of Leukemia, UT MD Anderson Cancer Center, United States.
Department of Hematopathology, UT MD Anderson Cancer Center, United States.
出版信息
BBA Clin. 2016 Dec 20;7:36-40. doi: 10.1016/j.bbacli.2016.12.002. eCollection 2017 Jun.
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation with macrophage and T-cell infiltration resulting in, multi organ damage. HLH may be primary or secondary in etiology. A high index of suspicion is essential for early diagnosis and treatment. Diagnostic criteria need to be refined and newer treatment options to be explored in order to improve survival especially in adult HLH and malignancy-associated HLH (M-HLH). We report a case of malignancy associated HLH (M-HLH) in adult treated on one of the only FDA-approved protocols for adult HLH to highlight the diagnostic and therapeutic challenges of this disease entity.
噬血细胞性淋巴组织细胞增生症（HLH）是一种严重免疫激活综合征，伴有巨噬细胞和T细胞浸润，导致多器官损害。HLH在病因上可能是原发性或继发性的。高度怀疑对于早期诊断和治疗至关重要。为了提高生存率，尤其是在成人HLH和恶性肿瘤相关HLH（M-HLH）中，诊断标准需要完善，新的治疗方案需要探索。我们报告一例成人恶性肿瘤相关HLH（M-HLH）病例，该病例采用了美国食品药品监督管理局（FDA）批准的仅有的成人HLH方案之一进行治疗，以突出这种疾病实体的诊断和治疗挑战。
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