Department of Hematology, Catholic Blood and Marrow Transplantation Center, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.
Department of Hematology, Catholic Blood and Marrow Transplantation Center, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
Haematologica. 2019 Feb;104(2):269-276. doi: 10.3324/haematol.2018.198655. Epub 2018 Sep 13.
Hemophagocytic lymphohistiocytosis is an overwhelming systemic inflammatory process that is life-threatening if not treated appropriately. We analyzed prognostic factors in patients with secondary hemophagocytic lymphohistiocytosis excluding malignancy. In this retrospective study, we analyzed 126 adult cases between 2001 and 2017. Treatment was based on dexamethasone with or without etoposide and cyclosporine. Patients who achieved a complete response by 4 weeks were defined as early stable responders, those who failed to achieve a complete response but showed continuous improvement until 8 weeks were defined as late responders, and those whose conditions waxed and waned until 8 weeks were defined as unstable responders. Patients with hemophagocytic lymphohistiocytosis caused by Epstein-Barr virus had a worse 5-year overall survival compared to those whose disease was secondary to autoimmune disease, other infections, or unknown causes (25.1% 82.4%, 78.7% and 55.5%, respectively; <0.001). We observed that the overall response rate at 4 weeks was similar, but decreased at 8 weeks in the Epstein-Barr virus subgroup from 75.5% to 51.0%, and finally decreased to 30.6%. Multivariate analysis revealed that 8-week treatment response was the most relevant factor for overall survival. Excluding 8-week response, the presence of Epstein-Barr virus, old age, hyperferritinemia, and thrombocytopenia were associated with poor survival. We established a prognostic model with the parameters: low-risk (score 0-1), intermediate-risk (score 2), and high-risk (score ≥3). These groups had 5-year overall survival rates of 92.1%, 36.8%, and 18.0%, respectively (<0.001). We found that 8-week treatment response was a good predictor for overall survival, and that Epstein-Barr virus, old age, thrombocytopenia, and hyperferritinemia were associated with poor survival outcomes. Physicians should take care to identify high-risk patients for appropriate treatment strategies.
噬血细胞性淋巴组织细胞增生症是一种危及生命的全身性炎症反应，如果治疗不当，可能会导致严重后果。本研究旨在分析排除恶性肿瘤的继发性噬血细胞性淋巴组织细胞增生症患者的预后因素。本回顾性研究分析了 2001 年至 2017 年间的 126 例成年患者。治疗方案为地塞米松联合依托泊苷和环孢素。4 周时达到完全缓解的患者定义为早期稳定缓解者，4 周时未达到完全缓解但持续改善至 8 周的患者定义为晚期缓解者，8 周时病情反复的患者定义为不稳定缓解者。与自身免疫性疾病、其他感染或原因不明的噬血细胞性淋巴组织细胞增生症相比，由 EBV 引起的噬血细胞性淋巴组织细胞增生症患者的 5 年总生存率较差（分别为 25.1%、82.4%、78.7%和 55.5%；<0.001）。我们观察到，4 周时的总体缓解率相似，但 EBV 亚组的缓解率在 8 周时从 75.5%下降至 51.0%，最终下降至 30.6%。多因素分析显示，8 周时的治疗反应是总生存的最相关因素。排除 8 周时的反应后，EBV 的存在、年龄较大、铁蛋白升高和血小板减少与较差的生存相关。我们建立了一个预后模型，参数包括：低危（评分 0-1）、中危（评分 2）和高危（评分≥3）。这些组的 5 年总生存率分别为 92.1%、36.8%和 18.0%（<0.001）。我们发现，8 周时的治疗反应是总生存的良好预测指标，而 EBV、年龄较大、血小板减少和铁蛋白升高与较差的生存结局相关。医生应注意识别高危患者，以便采取适当的治疗策略。
