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异基因造血干细胞移植后 PTLD 的频率、特征和结局:来自西班牙血液和骨髓移植组(GETH)的一项多中心研究。

Frequency, characteristics, and outcome of PTLD after allo-SCT: A multicenter study from the Spanish group of blood and marrow transplantation (GETH).

机构信息

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.

出版信息

Eur J Haematol. 2019 Jun;102(6):465-471. doi: 10.1111/ejh.13226. Epub 2019 Apr 10.

Abstract

UNLABELLED

Post-transplant lymphoproliferative disorder (PTLD) is an infrequent complication of allogeneic stem cell transplant (allo-SCT).

AIMS

To estimate the frequency and management of PTLD in Spain and to identify prognostic factors influencing outcomes.

METHODS

Multicenter, retrospective analysis of allo-SCT performed in 14 transplant units over a 15-year period.

RESULTS

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.

CONCLUSIONS

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.

摘要

未注明

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。

目的

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。

方法

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。

结果

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。

结论

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。

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