Schram Alison M, Comstock Paige, Campo Meghan, Gorovets Daniel, Mullally Ann, Bodio Kelly, Arnason Jon, Berliner Nancy
Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
Department of Medicine, Beth Israel Deaconess Medical Center, Boston, MA, USA.
Br J Haematol. 2016 Feb;172(3):412-9. doi: 10.1111/bjh.13837. Epub 2015 Nov 5.
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 53 years (range 18-77 years) 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 32·2 months, 46 patients had died (69%). The median overall survival was 4 months (95% CI: 0·0-10·2 months). Patients with malignancy had a worse prognosis compared to those without (median survival 2·8 months versus 10·7 months, P = 0·007). HLH is a devastating disorder with a high mortality. Further research is needed to improve treatment and outcomes.
噬血细胞性淋巴组织细胞增生症（HLH）是一种免疫激活失控的综合征，在过去十年中受到越来越多的关注。虽然传统上认为它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，但现在成人获得性HLH已得到广泛认可。这在恶性肿瘤、感染或风湿性疾病的背景下经常出现。我们对3个三级医疗中心进行了回顾性研究，确定了68例成人HLH患者。平均年龄为53岁（范围18 - 77岁），43例为男性（63%）。潜在疾病包括33例（49%）恶性肿瘤、22例（33%）感染、19例（28%）自身免疫性疾病和15例（22%）特发性HLH。患者接受了针对疾病的治疗和免疫调节剂治疗。中位随访32.2个月后，46例患者死亡（69%）。中位总生存期为4个月（95%CI：0.0 - 10.2个月）。与无恶性肿瘤的患者相比，有恶性肿瘤的患者预后更差（中位生存期2.8个月对10.7个月，P = 0.007）。HLH是一种具有高死亡率的毁灭性疾病。需要进一步研究以改善治疗和预后。
