成人的噬血细胞淋巴组织细胞病:一个未被认识的实体

BBA clinical Pub Date : 2017-06-01 DOI:10.1016/j.bbacli.2016.12.002

Abdul Rashid Shah , Tariq Muzzafar , Rita Assi , Dawid Schellingerhout , Zeev Estrov , Gevorg Tamamyan , Hagop Kantarjian , Naval Daver

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引用次数: 13

摘要

噬血细胞性淋巴组织细胞增多症(HLH)是一种严重的免疫激活综合征，巨噬细胞和t细胞浸润导致多器官损伤。HLH的病因可为原发性或继发性。高度的怀疑指数对于早期诊断和治疗至关重要。诊断标准需要改进，新的治疗方案需要探索，以提高生存率，特别是在成人HLH和恶性肿瘤相关的HLH (M-HLH)。我们报告一例恶性相关HLH (M-HLH)的成人病例，该病例采用fda批准的唯一成人HLH治疗方案之一，以突出该疾病实体的诊断和治疗挑战。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Hemophagocytic lymphohistiocytosis in adults: An under recognized entity

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.

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BBA clinical

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