Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research Karaikal, PUDUCHERRY, INDIA.
Turk Patoloji Derg. 2022;38(1):1-8. doi: 10.5146/tjpath.2021.01536.
Thrombotic microangiopathy (TMA) is often first detected on a renal biopsy performed for renal manifestations. Apart from hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura, there are various secondary conditions associated with TMA. This study analyzes the clinico-pathological spectrum, etiological factors and renal outcome of TMA diagnosed on renal biopsy.
A retrospective evaluation of renal biopsies for TMA over 5.5 years was performed. Clinical and laboratory data was collected from patient records.
A total of 40 biopsies from 39 patients showed TMA comprising 33 native and 7 transplant biopsies. Malignant hypertension (n=13) was the most common etiology in native biopsies followed by postpartum TMA (n=7), atypical HUS (aHUS) (n=7), and lupus nephritis (n=6). TMA in transplant biopsies was due to acute rejection (n=4) and CNI toxicity (n=3). Serum creatinine was high in most patients (mean 5.6 + 2.5 mg/ dl). aHUS showed the highest mean LDH levels and the lowest average platelet counts. Renal biopsies in malignant hypertension and postpartum TMA showed isolated arterial changes while aHUS and lupus nephritis showed both glomerular and arterial involvement. Postpartum TMA and aHUS had poor renal outcome requiring renal replacement therapy.
Most postpartum TMA and aHUS had systemic features of TMA while malignant hypertension and lupus nephritis showed 'isolated renal TMA'. This emphasizes the importance of careful evaluation of renal biopsies even in the absence of systemic features of TMA.
血栓性微血管病(TMA)通常首先在因肾脏表现而进行的肾活检中发现。除溶血尿毒综合征(HUS)和血栓性血小板减少性紫癜外,还有各种与 TMA 相关的继发性疾病。本研究分析了肾活检诊断为 TMA 的临床病理谱、病因因素和肾脏预后。
对 5.5 年内进行的肾活检 TMA 进行回顾性评估。从患者记录中收集临床和实验室数据。
39 名患者的 40 个活检显示 TMA,包括 33 个原发性和 7 个移植活检。原发性活检中最常见的病因是恶性高血压(n=13),其次是产后 TMA(n=7)、非典型 HUS(aHUS)(n=7)和狼疮性肾炎(n=6)。移植活检中的 TMA 是由急性排斥反应(n=4)和 CNI 毒性(n=3)引起的。大多数患者的血清肌酐升高(平均 5.6+2.5mg/dl)。aHUS 显示出最高的平均 LDH 水平和最低的平均血小板计数。恶性高血压和产后 TMA 的肾活检显示孤立性动脉变化,而 aHUS 和狼疮性肾炎则显示肾小球和动脉均受累。产后 TMA 和 aHUS 的肾脏预后不良,需要肾脏替代治疗。
大多数产后 TMA 和 aHUS 具有 TMA 的全身特征,而恶性高血压和狼疮性肾炎则表现为“孤立性肾脏 TMA”。这强调了即使在没有 TMA 的全身特征的情况下,仔细评估肾活检的重要性。