Lorenz Fryderyk, Klimkowska Monika, Pawłowicz Ewa, Bulanda Brustad Agnes, Erlanson Martin, Machaczka Maciej
a Department of Radiation Sciences, Section of Hematology , Umeå University , Umeå , Sweden.
b Department of Clinical Pathology and Cytology , Karolinska University Hospital Huddinge , Stockholm , Sweden.
Leuk Lymphoma. 2018 Aug;59(8):1840-1850. doi: 10.1080/10428194.2017.1403018. Epub 2018 Jan 3.
Hemophagocytic lymphohistiocytosis (HLH) is an underdiagnosed but life-threatening syndrome of hyperinflammation often occurring in adults with hematological malignancies (hM-HLH). The aim of the study was to describe clinical characteristics, therapy response, and outcome of adults with hM-HLH. The study included 51 adults with hM-HLH aged 23-84 years. Hyperferritinemia ≥500 µg/L was present in 96% of patients. The serum concentration of sIL-2Rα ≥ 2400 U/mL was revealed in 94% of patients. Twenty-three patients (45%) responded to therapy and achieved remission of HLH. The probability of overall survival (OS) at 6, 12, 24, and 60 months after HLH diagnosis were 42, 20, 15, and 15%, respectively. Patients with HLH during chemotherapy showed longer OS (median 124 days) than the patients who had HLH solely attributed to malignancy (median 65 days), but this difference was not statistically significant. Awareness of HLH in lymphoid and myeloid malignancies is crucial for improved survival.
噬血细胞性淋巴组织细胞增生症（HLH）是一种常发生于患有血液系统恶性肿瘤的成人（hM-HLH）中的炎症反应过度且诊断不足但危及生命的综合征。本研究的目的是描述hM-HLH成人患者的临床特征、治疗反应和预后。该研究纳入了51例年龄在23至84岁之间的hM-HLH成人患者。96%的患者存在高铁蛋白血症≥500µg/L。94%的患者血清sIL-2Rα浓度≥2400U/mL。23例患者（45%）对治疗有反应并实现了HLH缓解。HLH诊断后6个月、12个月、24个月和60个月的总生存率（OS）分别为42%、20%、15%和15%。化疗期间发生HLH的患者的OS（中位数124天）比单纯由恶性肿瘤导致HLH的患者（中位数65天）更长，但这种差异无统计学意义。认识到淋巴和髓系恶性肿瘤中的HLH对于提高生存率至关重要。
