Divisions of Nephrology and Hypertension, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.
Blood. 2011 Aug 11;118(6):1452-62. doi: 10.1182/blood-2011-02-321315. Epub 2011 May 19.
Transplantation-associated thrombotic microangiopathy (TA-TMA) is a challenging diagnosis after hematopoietic stem cell transplantation. Although endothelial injury represents the final common pathway of disease, the exact pathophysiology of TA-TMA remains unclear. Potential causes include infections, chemotherapy, radiation, and calcineurin inhibitors. Recent literature addresses the roles of cytokines, graft-versus-host disease, the coagulation cascade, and complement in the pathogenesis of TA-TMA. Current diagnostic criteria are unsatisfactory, because patients who have received a transplant can have multiple other reasons for the laboratory abnormalities currently used to diagnose TA-TMA. Moreover, our lack of understanding of the exact mechanism of disease limits the development and evaluation of potential treatments. Short- and long-term renal complications contribute to TA-TMA's overall poor prognosis. In light of these challenges, future research must validate novel markers of disease to aid in early diagnosis, guide current and future treatments, prevent long-term morbidity, and improve outcomes. We focus on TA-TMA as a distinct complication of hematopoietic stem cell transplantation, emphasizing the central role of the kidney in this disease.
移植相关血栓性微血管病（TA-TMA）是造血干细胞移植后的一个具有挑战性的诊断。尽管内皮细胞损伤代表了疾病的最终共同途径，但 TA-TMA 的确切病理生理学仍不清楚。潜在的原因包括感染、化疗、放疗和钙调神经磷酸酶抑制剂。最近的文献探讨了细胞因子、移植物抗宿主病、凝血级联和补体在 TA-TMA 发病机制中的作用。目前的诊断标准并不令人满意，因为接受过移植的患者可能有多种其他原因导致目前用于诊断 TA-TMA 的实验室异常。此外，我们对疾病的确切机制缺乏了解，限制了潜在治疗方法的开发和评估。短期和长期的肾脏并发症导致 TA-TMA 的整体预后不良。鉴于这些挑战，未来的研究必须验证疾病的新标志物，以帮助早期诊断、指导当前和未来的治疗、预防长期发病和改善预后。我们专注于 TA-TMA 作为造血干细胞移植的一种独特并发症，强调肾脏在这种疾病中的核心作用。
