Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
Cell Transplant. 2021 Jan-Dec;30:9636897211057077. doi: 10.1177/09636897211057077.
Hemophagocytic lymphohistiocytosis (HLH) is a severe or even fatal inflammatory status. Lymphoma associated hemophagocytic lymphohistiocytosis (LAHS) is a kind of secondary HLH (sHLH). It suffers the worst outcome among sHLH. Allo-HSCT is often considered necessary. Autologous stem cell transplantation (auto-SCT) is widely used in the treatment of lymphoma, especially for high-risk NHL. There have been no clinical reports on the use of auto-SCT in LAHS in the past 20 years.
We retrospectively evaluated 12 LAHS patients who received auto-SCT at our center from January 2013 to January 2020. Follow-up started at the date of LAHS diagnosis and ended at the date of death or last examination. Overall survival (OS) was calculated from the diagnosis of HLH to death of any cause.
The median period between diagnosis and auto-SCT is 6.7 months. All 12 patients achieved remission after transplantation. Follow-up to 1 January 2021, 8 patients remained disease-free, 4 patients relapsed and 2 of them died eventually. The median follow-up time is 20.9 months, and the median overall survival time has not been reached yet. The 3-year OS rates was 71%. Compared with LAHS patients who did not undergo transplantation during the same period (median OS time is 3.4 months), patients who underwent auto-SCT had a significantly better prognosis (=0.001). Even if the lymphoma reaches CR after treatment, auto-SCT still provides a better prognosis compared to CR patients without transplantation (=0.037). Compared with lymphoma patients without HLH who underwent auto-SCT during the same period, they had a similar prognosis (=0.350).
LAHS, as a common type in secondary HLH, may have a better prognosis after removing the trigger of HLH. In this study, the autologous transplantation in LAHS can significantly improve the prognosis, and provide LAHS a similar prognosis as high-risk lymphoma without HLH.
噬血细胞性淋巴组织细胞增生症（HLH）是一种严重甚至致命的炎症状态。淋巴瘤相关性噬血细胞性淋巴组织细胞增生症（LAHS）是一种继发性 HLH（sHLH）。它是 sHLH 中预后最差的一种。异基因造血干细胞移植（allo-HSCT）通常被认为是必要的。自体造血干细胞移植（auto-SCT）广泛应用于淋巴瘤的治疗，尤其是高危 NHL。过去 20 年，LAHS 患者使用 auto-SCT 尚无临床报道。
我们回顾性评估了 2013 年 1 月至 2020 年 1 月期间在我院接受 auto-SCT 的 12 例 LAHS 患者。随访自 LAHS 诊断开始，至任何原因死亡或最后一次检查结束。总体生存（OS）时间从 HLH 诊断开始计算至任何原因死亡。
诊断至 auto-SCT 的中位时间为 6.7 个月。所有 12 例患者移植后均达到缓解。截至 2021 年 1 月 1 日的随访结果显示，8 例患者无疾病生存，4 例复发，其中 2 例最终死亡。中位随访时间为 20.9 个月，中位总生存时间尚未达到。3 年 OS 率为 71%。与同期未移植的 LAHS 患者相比（中位 OS 时间为 3.4 个月），接受 auto-SCT 的患者预后明显更好（=0.001）。即使治疗后淋巴瘤达到完全缓解（CR），与未接受移植的 CR 患者相比，auto-SCT 仍能提供更好的预后（=0.037）。与同期无 HLH 的淋巴瘤患者相比，接受 auto-SCT 的患者预后相似（=0.350）。
LAHS 作为继发性 HLH 的常见类型，在去除 HLH 的诱因后可能有更好的预后。在这项研究中，LAHS 中的自体移植可显著改善预后，并为 LAHS 提供与无 HLH 的高危淋巴瘤相似的预后。
