Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University, 54 Kawaharacho, Shogoin, Sakyo-ku, Kyoto, 606-8507, Japan.
Ann Hematol. 2020 Jul;99(7):1565-1573. doi: 10.1007/s00277-020-04084-5. Epub 2020 May 20.
The number of patients who are administered immunosuppressive agents has been increasing. Accordingly, more patients face higher risks for developing immunodeficiency-associated lymphoproliferative disorders (LPD). Although immunodeficiency-associated LPD are distinct from other lymphoid neoplasms in terms of their immunocompromised backgrounds, little is known about the impact of lymphopenia at diagnosis on survival in patients with these LPD. Seventy-one immunodeficiency-associated LPD in Kyoto University Hospital (post-transplant LPD (PTLD), n = 26; other iatrogenic immunodeficiency-associated LPD, n = 45) were reviewed and analyzed. The median age at diagnosis was 63 years (range, 3-83). Diffuse large B cell lymphoma was the most common subtype (n = 33), followed by Hodgkin lymphoma (n = 12), B cell monomorphic LPD not specified (n = 11), and polymorphic LPD or early-phase diseases (n = 15). The median follow-up period for survivors was 2.5 years and overall survival (OS) and progression-free survival (PFS) at 2.5 years were 75% and 67%, respectively. Multivariate analysis showed that lymphopenia (≤ 800/μL) at diagnosis predicted inferior OS (HR, 3.72; P = 0.043) and PFS (HR, 3.82; P = 0.012). Serum albumin values also strongly affected OS (> 3.18 g/dL vs. ≤ 3.18 g/dL; HR, 0.21; P = 0.010) and PFS (HR, 0.26; P = 0.013). Lymphopenia at diagnosis is suggested to predict inferior OS and PFS in patients with immunodeficiency-associated LPDs. Immunocompromised status might affect disease progression in these distinct lymphoid neoplasms growing under immunocompromised backgrounds.
接受免疫抑制药物治疗的患者数量一直在增加。因此，更多的患者面临更高的免疫缺陷相关淋巴增生性疾病（LPD）发病风险。虽然免疫缺陷相关 LPD 与其免疫抑制背景下的其他淋巴肿瘤在发病机制上有所不同，但对于这些 LPD 患者在诊断时的淋巴细胞减少对生存的影响知之甚少。对京都大学医院的 71 例免疫缺陷相关 LPD（移植后 LPD（PTLD），n=26；其他医源性免疫缺陷相关 LPD，n=45）进行了回顾性分析。诊断时的中位年龄为 63 岁（范围 3-83 岁）。弥漫性大 B 细胞淋巴瘤是最常见的亚型（n=33），其次是霍奇金淋巴瘤（n=12）、非特指的 B 细胞单形性 LPD（n=11）和多形性 LPD 或早期疾病（n=15）。幸存者的中位随访时间为 2.5 年，2.5 年的总生存率（OS）和无进展生存率（PFS）分别为 75%和 67%。多变量分析显示，诊断时的淋巴细胞减少（≤800/μL）预示着 OS（HR，3.72；P=0.043）和 PFS（HR，3.82；P=0.012）更差。血清白蛋白值也强烈影响 OS（>3.18 g/dL 与≤3.18 g/dL；HR，0.21；P=0.010）和 PFS（HR，0.26；P=0.013）。诊断时的淋巴细胞减少提示免疫缺陷相关 LPD 患者的 OS 和 PFS 更差。免疫抑制状态可能会影响这些在免疫抑制背景下生长的不同淋巴肿瘤的疾病进展。
