Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan.
Pathol Int. 2011 Sep;61(9):518-27. doi: 10.1111/j.1440-1827.2011.02704.x. Epub 2011 Jul 12.
Thrombotic microangiopathy (TMA) is a major complication after hematopoietic stem cell transplantation (HSCT). In this study, we examined the clinical and pathologic features of 2 patients and 5 autopsy cases with HSCT-associated renal TMA to clarify the association between graft-versus-host disease (GVHD) and renal TMA. The median interval between HSCT and renal biopsy or autopsy was 7 months (range 3-42 months). Clinically, acute and chronic GVHD occurred in 7 and 4 patients, respectively. Clinical evidence for TMA was detected in 2 patients, while chronic kidney disease developed in all patients. The main histopathological findings were diffuse endothelial injury in glomeruli, peritubular capillaries (PTCs), and small arteries. In addition, all cases showed glomerulitis, renal tubulitis, and peritubular capillaritis with infiltration of CD3+ T cells and TIA-1+ cytotoxic cells, suggesting that GVHD occurred during the development of TMA. Diffuse and patchy C4d deposition was noted in glomerular capillaries and PTCs, respectively, in 2 biopsy and 2 autopsy cases, suggesting the involvement of antibody-mediated renal endothelial injury in more than 50% of renal TMA cases. In conclusion, the kidney is a potential target of chronic GVHD that may induce the development of HSCT-associated TMA. Importantly, some cases are associated with chronic humoral GVHD.
血栓性微血管病（TMA）是造血干细胞移植（HSCT）后的主要并发症。在这项研究中，我们检查了 2 例患者和 5 例 HSCT 相关肾 TMA 尸检病例的临床和病理特征，以阐明移植物抗宿主病（GVHD）与肾 TMA 之间的关系。HSCT 与肾活检或尸检之间的中位间隔为 7 个月（范围 3-42 个月）。临床上，分别有 7 例和 4 例患者发生急性和慢性 GVHD。2 例患者检测到 TMA 的临床证据，而所有患者均出现慢性肾脏病。主要的组织病理学发现是肾小球、肾小管周围毛细血管（PTC）和小动脉的弥漫性内皮损伤。此外，所有病例均表现为肾小球肾炎、肾小管炎和 PTC 炎，伴有 CD3+T 细胞和 TIA-1+细胞毒性细胞浸润，提示 GVHD 发生在 TMA 发展过程中。2 例活检和 2 例尸检病例分别在肾小球毛细血管和 PTC 中观察到弥漫性和斑片状 C4d 沉积，提示 50%以上的肾 TMA 病例存在抗体介导的肾内皮损伤。总之，肾脏是慢性 GVHD 的潜在靶器官，可能导致 HSCT 相关 TMA 的发生。重要的是，一些病例与慢性体液性 GVHD 有关。
