Department of Hematology of the: Hospital de la Santa Creu I Sant Pau, Biomedical Research Institute (IIB Sant-Pau), Autonomous University of Barcelona, Barcelona, Spain.
HU. Marqués de Valdecilla, Santander, Spain.
Eur J Haematol. 2019 Jun;102(6):465-471. doi: 10.1111/ejh.13226. Epub 2019 Apr 10.
Post-transplant lymphoproliferative disorder (PTLD) is an infrequent complication of allogeneic stem cell transplant (allo-SCT).
To estimate the frequency and management of PTLD in Spain and to identify prognostic factors influencing outcomes.
Multicenter, retrospective analysis of allo-SCT performed in 14 transplant units over a 15-year period.
102 PTLD were diagnosed among 12 641 allo-SCT, leading to an estimated frequency of 0.8%. PTLD was diagnosed at a median of 106 days after SCT. Eighty-seven cases (85%) were diagnosed between 2007 and 2013. At diagnosis, 22% and 17% of the patients had gastrointestinal tract and CNS involvement. Eighty-seven (85%) received rituximab treatment, alone or in combination with immunosuppression reduction, with an ORR of 50.6%. With a median follow-up for survivors of 58 months, the 2-year overall survival (OS) was 33% and the PTLD-related mortality 45%. Age ≥ 40 years, malignant underlying disease, non-response to rituximab, and severe thrombocytopenia or lymphocytopenia at PTLD diagnosis were associated with worse overall survival.
Only a small proportion of allografted patients were diagnosed a PTLD. Its clinical course was highly aggressive, and prognosis poor, especially in those failing rituximab. The prognostic impact found of the platelet, and lymphocyte count at diagnosis requires further confirmation.
同种异体干细胞移植（allo-SCT）后发生的淋巴增生性疾病（PTLD）是一种罕见的并发症。
估计西班牙同种异体干细胞移植后发生 PTLD 的频率和管理方法，并确定影响结局的预后因素。
对 14 个移植单位在 15 年内进行的 allo-SCT 进行了多中心回顾性分析。
在 12641 例 allo-SCT 中诊断出 102 例 PTLD，估计发生率为 0.8%。PTLD 在 SCT 后中位数 106 天诊断。87 例（85%）在 2007 年至 2013 年期间诊断。诊断时，22%和 17%的患者有胃肠道和中枢神经系统受累。87 例（85%）接受利妥昔单抗治疗，单独或与免疫抑制减少联合治疗，客观缓解率为 50.6%。在幸存者的中位随访 58 个月后，2 年总生存率（OS）为 33%，PTLD 相关死亡率为 45%。年龄≥40 岁、恶性基础疾病、对利妥昔单抗无反应以及在 PTLD 诊断时出现严重血小板减少或淋巴细胞减少与总体生存率较差相关。
只有一小部分接受同种异体移植的患者被诊断为 PTLD。其临床病程具有高度侵袭性，预后较差，尤其是在那些对利妥昔单抗无反应的患者中。在诊断时发现血小板和淋巴细胞计数的预后影响需要进一步证实。
