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Transplant-associated thrombotic microangiopathy: Incidence, prognostic factors, morbidity, and mortality in allogeneic hematopoietic cell transplantation

机译:移植相关的血栓形成微观病症:异种造血细胞移植的发病率,预后因素,发病率和死亡率

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摘要

Renewed interest has emerged in transplant-associated thrombotic microangiopathy (TA-TMA) with novel prognostic, diagnostic, and treatment algorithms. We aimed to investigate the incidence, prognostic factors, morbidity, and mortality of TA-TMA in allogeneic hematopoietic cell transplantation (HCT) recipients. We enrolled consecutive HCT recipients (1990-2017). Among 758 patients, 116 (15.5%) were diagnosed with TA-TMA. In the multivariate analysis, TBI-based conditioning, viral infections, acute and chronic GVHD remained independent predictors of TA-TMA. With a median follow-up of 23 (range 0.1-329) months, TA-TMA resulted in significantly lower overall survival (OS). In the multivariate analysis, TA-TMA remained an independent predictor of OS, along with relapse, acute, and chronic GVHD. Among 116 TA-TMA patients, 70 developed renal (56) and/or neurologic (26) dysfunction that would be necessary for TA-TMA diagnosis according to the Bone Marrow Transplant Clinical Trials Network criteria. TA-TMA patients with renal dysfunction showed increased rates of acute GVHD, but no difference in OS compared to patients without renal dysfunction. However, neurologic dysfunction resulted in significantly lower OS. In conclusion, TA-TMA is associated with increased morbidity and mortality in allogeneic transplant recipients. Successful prevention and treatment strategies of infections and GVHD need to be timely employed to improve survival in this complex setting.
机译:具有新型预后,诊断和治疗算法的移植相关血栓性微动病变(TA-TMA)中出现了更新的兴趣。我们旨在探讨同种异体造血细胞移植(HCT)受者的TA-TMA的发病,预后因素,发病率和死亡率。我们注册了连续的HCT接受者(1990-2017)。在758名患者中,116名(15.5%)被诊断出TA-TMA。在多变量分析中,基于TBI的调理,病毒感染,急性和慢性GVHD仍然是TA-TMA的独立预测因子。中位后续23(范围0.1-329)个月,TA-TMA导致总体存活率显着降低(OS)。在多变量分析中,TA-TMA仍然是OS的独立预测因子,以及复发,急性和慢性GVHD。在116例TA-TMA患者中,70名发育肾(56)和/或神经系统(26)功能障碍,这是根据骨髓移植临床试验网络标准的TA-TMA诊断所必需的。 TA-TMA肾功能紊乱患者显示出急性GVHD的速率增加,但与没有肾功能不全的患者相比,OS没有差异。然而,神经功能障碍导致OS显着降低。总之,TA-TMA与同种异体移植受者的发病率和死亡率增加有关。需要及时使用感染和GVHD的成功预防和治疗策略,以改善这种复杂环境中的存活。

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