A consensus review on malignancy-associated hemophagocytic lymphohistiocytosis in adults.
A consensus review on malignancy-associated hemophagocytic lymphohistiocytosis in adults.
复制标题
成人对恶性相关的淋巴淋巴虫组织细胞增多症的共识综述。
DOI：
10.1002/cncr.30826
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发表时间：
2017-09-01
期刊：
影响因子：
6.2
通讯作者：
Kantarjian HM
中科院分区：
文献类型：
--
作者：
Daver N;McClain K;Allen CE;Parikh SA;Otrock Z;Rojas-Hernandez C;Blechacz B;Wang S;Minkov M;Jordan MB;La Rosée P;Kantarjian HM
噬血细胞性淋巴组织细胞增生症（HLH）是一种严重的免疫激活和失调综合征，导致极端的，往往危及生命的炎症。HLH在儿科人群中得到了很好的认可，目前大多数诊断和治疗指南都是基于儿科HLH。最近，人们认识到成人中存在HLH，尤其是继发于潜在的风湿性、感染性或恶性疾病引起的免疫失调。在这篇综述中，我们专注于恶性相关的HLH（M-HLH）。在M-HLH中，可能的发病机制包括严重炎症、肿瘤细胞的持续抗原刺激以及由于化疗、造血干细胞移植或感染而导致的免疫稳态丧失。以前认为罕见，M-HLH可能发生在高达1%的血液恶性肿瘤患者中。在大多数已发表的研究中，M-HLH经常被漏诊或诊断较晚，并且与不到2个月的中位生存期相关。识别成人M-HLH特异性的临床和实验室特征可能有助于早期发现、咨询HLH专家并进行干预。通过T淋巴细胞溶解剂和免疫抑制剂的最佳组合以及基于儿科经验的新型药物的结合来改善成人M-HLH的管理，有望改善成人M-HLH的结局。
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation and dysregulation resulting in extreme and often life-threatening inflammation. HLH has been well recognized in pediatric populations and most current diagnostic and therapeutic guidelines are based on pediatric HLH. Recently there has been recognition of HLH in adults, especially secondary to immune deregulation by an underlying rheumatologic, infectious, or malignant condition. In this review we focus on malignancy-associated HLH (M-HLH). In M-HLH possible mechanisms of pathogenesis include severe inflammation, persistent antigen stimulation by the tumor cells, and loss of immune homeostasis due to chemotherapy, hematopoietic stem cell transplant or infection. Previously considered rare, M-HLH may occur in up to 1% of patients with hematologic malignancies. M-HLH is often missed or diagnosed late in most published studies, and associated with a poor median survival of less than 2 months. Identification of the clinical and laboratory features specific to M-HLH in adults may allow early detection, consultation with HLH experts, and intervention. Improved management of adult M-HLH with optimal combinations of T-lympholytic and immunosuppressive agents and incorporation of novel agents based on the pediatric experience will hopefully improve outcomes in M-HLH in adults.
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影响因子：
3.5
作者：
Ho, Caleb;Yao, Xiaopan;Xu, Mina L.
通讯作者：
Xu, Mina L.
影响因子：
14.2
作者：
Humblet-Baron, Stephanie;Franckaert, Dean;Liston, Adrian
通讯作者：
Liston, Adrian
影响因子：
3.7
作者：
Hejblum G;Lambotte O;Galicier L;Coppo P;Marzac C;Aumont C;Fardet L
通讯作者：
Fardet L
影响因子：
6.5
作者：
AKASHI, K;HAYASHI, S;NIHO, Y
通讯作者：
NIHO, Y
DOI：
10.1016/s0889-8588(05)70521-9
发表时间：
1998-04-01
影响因子：
2.4
作者：
Janka, G;Imashuku, S;Henter, JI
通讯作者：
Henter, JI