Kümpers P, Herrmann A, Lotz J, Mengel M, Schwarz A
Department of Nephrology, Medical School Hannover, Carl-Neuberg-Strasse 1, 30625 Hannover, Germany.
Clin Nephrol. 2006 Sep;66(3):210-3. doi: 10.5414/cnp66210.
Acute renal failure is a known complication during hemolytic crisis in paroxysmal nocturnal hemoglobinuria (PNH). However, chronic renal failure is rare despite the well-known spectacular hemosiderosis of the kidneys due to chronic hemolysis. Here, we report about a 74-year-old man with PNH who developed acute on chronic renal failure after an episode of intercurrent urinary tract infection and subsequent hemolytic crisis. Mild chronic hemolysis, well-documented over the past decade, had long been considered the cause of a constantly declining glomerular filtration rate. Accordingly, magnetic resonance imaging during admission demonstrated marked siderosis of both kidneys, supporting the hypothesis that chronic renal failure (CRF) was likewise related to PNH. However, a renal biopsy revealed acute tubular necrosis and distinct renal siderosis, as expected. Additionally, tubulointerstitial injury and global glomerular sclerosis, best classified as arterionephrosclerosis, were present. In retrospect, these findings were explained by a 15-year history of hypertension and a 4-year medication with cyclosporine. Careful diagnostic workup including a renal biopsy is mandatory, given a misleadingly suggestive correlation between chronic hemolysis and CRF. Chronic renal failure in PNH is a diagnosis of exclusion, even if radiologic evidence of heavy siderosis draws off the physician's attention.
急性肾衰竭是阵发性夜间血红蛋白尿（PNH）溶血危象期间已知的并发症。然而，尽管由于慢性溶血导致众所周知的明显肾脏含铁血黄素沉着症，但慢性肾衰竭却很罕见。在此，我们报告一例74岁的PNH男性患者，在并发尿路感染及随后的溶血危象发作后发生了慢性肾衰竭基础上的急性肾衰竭。过去十年间有充分记录的轻度慢性溶血长期以来一直被认为是肾小球滤过率持续下降的原因。因此，入院时的磁共振成像显示双肾明显的含铁血黄素沉着，支持慢性肾衰竭（CRF）同样与PNH相关的假说。然而，肾活检显示为急性肾小管坏死及明显的肾脏含铁血黄素沉着，正如预期。此外，还存在肾小管间质损伤和以肾小动脉硬化症最佳分类的全球肾小球硬化。回顾来看，这些发现可由15年的高血压病史和4年的环孢素用药史来解释。鉴于慢性溶血与CRF之间存在误导性的提示性关联，包括肾活检在内的仔细诊断检查是必不可少的。即使严重含铁血黄素沉着的影像学证据吸引了医生的注意力，PNH中的慢性肾衰竭仍是一种排除性诊断。
