Puri V, Gandhi A, Sharma S
Department of Pathology, University College of Medical Sciences, New Delhi, India.
Indian J Nephrol. 2017 Jul-Aug;27(4):284-288. doi: 10.4103/0971-4065.202833.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, potentially life-threatening disease of blood, characterized by complement-induced intravascular hemolytic anemia and thrombosis. PNH can sometimes present directly with renal manifestations, without showing any hematological manifestation. It, therefore, becomes essential for clinicians and pathologists to be aware of the spectrum of renal changes in PNH. The aim of this study was to document the morphologic changes observed in renal biopsies in patients with PNH. This is an observational study. We report three cases that presented with acute or chronic renal insufficiency and were suspected as PNH on viewing their renal biopsy in light of their clinical and laboratory details. All the three cases were confirmed as PNH on the basis of flow cytometric analysis of CD55 and CD59. Renal biopsy in these patients showed a variety of morphologic changes, however the most consistent finding was moderate-to-heavy siderosis in their kidneys. PNH may be difficult to diagnose clinically and sometimes present directly with renal manifestations. It is, therefore, prudent for nephrologists and nephropathologists to be aware of the spectrum of renal changes in PNH.
阵发性睡眠性血红蛋白尿（PNH）是一种罕见的、后天获得性的、潜在危及生命的血液疾病，其特征为补体介导的血管内溶血和血栓形成。PNH有时可直接表现为肾脏症状，而无任何血液学表现。因此，临床医生和病理学家了解PNH肾脏变化的范围至关重要。本研究的目的是记录PNH患者肾活检中观察到的形态学变化。这是一项观察性研究。我们报告了3例表现为急性或慢性肾功能不全的病例，根据其临床和实验室检查结果，在查看肾活检时怀疑为PNH。根据CD55和CD59的流式细胞术分析，所有3例均确诊为PNH。这些患者的肾活检显示出多种形态学变化，但最一致的发现是肾脏中、重度含铁血黄素沉着。PNH临床诊断可能困难，有时可直接表现为肾脏症状。因此，肾病学家和肾病理学家了解PNH肾脏变化的范围是谨慎之举。
