Mubarak Muhammed, Raza Amber, Rashid Rahma, Sapna Fnu, Shakeel Shaheera
Department of Histopathology, Sindh Institute of Urology and Transplantation, Karachi 74200, Sindh, Pakistan.
Department of Nephrology, Sindh Institute of Urology and Transplantation, Karachi 74200, Sindh, Pakistan.
World J Transplant. 2024 Mar 18;14(1):90277. doi: 10.5500/wjt.v14.i1.90277.
Thrombotic microangiopathy (TMA) is an uncommon but serious complication that not only affects native kidneys but also transplanted kidneys. This review is specifically focused on post-transplant TMA (PT-TMA) involving kidney transplant recipients. Its reported prevalence in the latter population varies from 0.8% to 14% with adverse impacts on both graft and patient survival. It has many causes and associations, and the list of etiologic agents and associations is growing constantly. The pathogenesis is equally varied and a variety of patho genetic pathways lead to the development of microvascular injury as the final common pathway. PT-TMA is categorized in many ways in order to facilitate its management. Ironically, more than one causes are contributory in PT-TMA and it is often difficult to pinpoint one particular cause in an individual case. Pathologically, the hallmark lesions are endothelial cell injury and intravascular thrombi affecting the microvasculature. Early diagnosis and classification of PT-TMA are imperative for optimal outcomes but are challenging for both clinicians and pathologists. The Banff classification has addressed this issue and has developed minimum diagnostic criteria for pathologic diagnosis of PT-TMA in the first phase. Management of the condition is also challenging and still largely empirical. It varies from simple maneuvers, such as plasmapheresis, drug withdrawal or modification, or dose reduction, to lifelong complement blockade, which is very expensive. A thorough understanding of the condition is imperative for an early diagnosis and quick treatment when the treatment is potentially effective. This review aims to increase the awareness of relevant stakeholders regarding this important, potentially treatable but under-recognized cause of kidney allograft dysfunction.
血栓性微血管病(TMA)是一种罕见但严重的并发症,不仅会影响自体肾,也会影响移植肾。本综述专门聚焦于涉及肾移植受者的移植后TMA(PT-TMA)。据报道,其在后者人群中的患病率从0.8%到14%不等,对移植肾和患者存活均有不利影响。它有许多病因和相关因素,病因因子和相关因素的清单在不断增加。其发病机制同样多样,多种致病途径最终都会导致微血管损伤。PT-TMA有多种分类方式以利于其管理。具有讽刺意味的是,PT-TMA往往不止一个病因起作用,在个别病例中常常难以确定一个特定病因。病理上,标志性病变是影响微血管的内皮细胞损伤和血管内血栓形成。PT-TMA的早期诊断和分类对于获得最佳结果至关重要,但对临床医生和病理学家而言都具有挑战性。班夫分类法已解决了这一问题,并在第一阶段制定了PT-TMA病理诊断的最低诊断标准。该病症的管理也具有挑战性,且在很大程度上仍基于经验。其管理方式从简单操作,如血浆置换、停药或调整药物、或减少剂量,到终身补体阻断(这非常昂贵)不等。当治疗可能有效时,透彻了解该病症对于早期诊断和快速治疗至关重要。本综述旨在提高相关利益攸关方对这种重要的、可能可治疗但未得到充分认识的肾移植功能障碍病因的认识。