Haemophagocytic lymphohistiocytosis in adults: a multicentre case series over 7years
Haemophagocytic lymphohistiocytosis in adults: a multicentre case series over 7years
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DOI：
10.1111/bjh.13837
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发表时间：
2016-02-01
影响因子：
6.5
通讯作者：
Berliner, Nancy
中科院分区：
文献类型：
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作者：
Schram, Alison M.;Comstock, Paige;Berliner, Nancy
噬血细胞性淋巴组织细胞增多症 (HLH) 是一种免疫激活不受控制的综合征，在过去十年中受到越来越多的关注。尽管传统上认为 HLH 是一种由影响细胞毒性 T 细胞功能的突变引起的儿童家族性疾病，但现在已广泛认识到成人获得性 HLH。这种情况常见于恶性肿瘤、感染或风湿性疾病。我们对 3 个三级护理中心进行了回顾性审查，确定了 68 名患有 HLH 的成年人。平均年龄为53岁（范围18-77岁），其中男性43人（63%）。基础疾病包括 33 名患者 (49%) 患有恶性肿瘤、22 名患者 (33%) 患有感染、19 名患者 (28%) 患有自身免疫性疾病以及 15 名患者 (22%) 患有特发性 HLH。患者接受疾病特异性治疗和免疫调节剂治疗。中位随访 322 个月后，46 名患者死亡（69%）。中位总生存期为 4 个月（95% CI：00-102 个月）。与非恶性肿瘤患者相比，患有恶性肿瘤的患者预后较差（中位生存期为 28 个月 vs 107 个月，P=0007）。 HLH 是一种死亡率很高的毁灭性疾病。需要进一步研究来改善治疗和结果。
Haemophagocytic lymphohistiocytosis (HLH) is a syndrome of uncontrolled immune activation that has gained increasing attention over the past decade. Although classically known as a familial disorder of children caused by mutations that affect cytotoxic T-cell function, an acquired form of HLH in adults is now widely recognized. This is often seen in the setting of malignancy, infection or rheumatological disorders. We performed a retrospective review across 3 tertiary care centres and identified 68 adults with HLH. The average age was 53years (range 18-77years) and 43 were male (63%). Underlying disorders included malignancy in 33 patients (49%), infection in 22 (33%), autoimmune disease in 19 (28%) and idiopathic HLH in 15 (22%). Patients were treated with disease-specific therapy and immunomodulatory agents. After a median follow-up of 322months, 46 patients had died (69%). The median overall survival was 4months (95% CI: 00-102months). Patients with malignancy had a worse prognosis compared to those without (median survival 28months versus 107months, P=0007). HLH is a devastating disorder with a high mortality. Further research is needed to improve treatment and outcomes.