Department and Institution: Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
Cancer Biol Ther. 2021 Dec 2;22(10-12):598-606. doi: 10.1080/15384047.2021.1996139. Epub 2021 Nov 1.
Hemophagocytic lymphohistiocytosis (HLH) is a severe or even fatal inflammatory status caused by a hereditary or acquired immunoregulatory abnormality. Lymphoma-associated hemophagocytic lymphohistiocytosis (LAHS) is a kind of secondary HLH (sHLH). It suffers the worst outcome among sHLH. However, there is no standard treatment strategy. The argument mainly focuses on whether an HLH-directed or malignancy-directed approach should initially be adopted. Etoposide is one of the key drugs in HLH treatment and also shows activity in lymphomas. We sought to identify the importance of containing etoposide in the initial treatment of LAHS. 66 patients diagnosed with LAHS in our center during the three years were divided into two groups according to whether the initial treatment involved etoposide or lymphoma-directed chemotherapy without etoposide. The remission rate of the initial etoposide group (52 patients) is significantly better than that of the no initial etoposide group (14 patients) (73.1% vs. 42.9%, = .033). The two-month survival rate (79.8% vs. 46.8%, = .035) and overall survival (median survival time 25.8 w vs. 7.8 w, = .048) of the initial etoposide contained group is significantly better. Multivariate cox analysis revealed that for patients without EBV infection (37 cases), initial treatment with etoposide could significantly improve prognosis ( = .010, Exp(B) = 0.183), but for patients with positive EBV, it shows a tendency. Containing etoposide is beneficial in the initial treatment of LAHS, whether in the HLH-directed or lymphoma-directed strategy. It provides higher response rate, lower mortality rate, and better survival, especially for EBV negative patients.
噬血细胞性淋巴组织细胞增生症（HLH）是一种由遗传性或获得性免疫调节异常引起的严重甚至致命的炎症状态。淋巴瘤相关噬血细胞性淋巴组织细胞增生症（LAHS）是一种继发性 HLH（sHLH）。它在 sHLH 中预后最差。然而，目前尚无标准的治疗策略。争论的主要焦点是最初应采用 HLH 定向还是恶性肿瘤定向方法。依托泊苷是 HLH 治疗的关键药物之一，在淋巴瘤中也具有活性。我们试图确定在 LAHS 的初始治疗中包含依托泊苷的重要性。我们中心在三年内诊断出的 66 例 LAHS 患者根据初始治疗是否涉及依托泊苷或不包含依托泊苷的淋巴瘤定向化疗分为两组。初始依托泊苷组（52 例）的缓解率明显优于无初始依托泊苷组（14 例）（73.1%对 42.9%， =.033）。初始依托泊苷组的两个月生存率（79.8%对 46.8%， =.035）和总生存率（中位生存时间 25.8 w 对 7.8 w， =.048）明显更好。多变量 Cox 分析显示，对于无 EBV 感染的患者（37 例），初始依托泊苷治疗可显著改善预后（ =.010，Exp（B）= 0.183），但对于 EBV 阳性患者，这种趋势不明显。包含依托泊苷在 LAHS 的初始治疗中是有益的，无论是在 HLH 定向还是淋巴瘤定向策略中。它提供了更高的反应率、更低的死亡率和更好的生存率，特别是对于 EBV 阴性患者。
