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Recurrent Acute Renal Failure in a Patient with Aplastic Anemia-Paroxysmal Nocturnal Hemoglobinuria Syndrome: A Case Report

一再生不良性貧血-陣發性夜間血色素尿症病人發生反覆性急性腎衰竭-病例報告

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摘要


急性腎衰竭是再生不良性貧血-陣發性夜間血色素尿症病人發生溶血急症的一個併發症。本文報告一例再生不良性貧血病患發生反覆性急性腎衰竭,證實和陣發性夜間血色素尿症相關。一位四十六歲再生不良性貧血女性患者在泌尿道感染後,因為寡尿及暗色尿而住院,診斷為陣發性夜間血色素尿症合併急性腎衰竭,並因為無尿急性腎衰竭而接受九次的血液透析,之後腎臟功能幾乎完全回復。不幸在四個月後,病人再度因為血色素尿及腎臟功能惡化而住院。腎臟切片檢查顯示急性腎小管壞死,血鐵質沉積於近端腎小管細胞並且呈陽性尿酸鹽染色,無血管栓塞。經過適當的輸液治療、利尿劑及重碳酸鹽處理,腎臟功能回復。這是一起有關再生不良性貧血病患在短時間內發生反覆性血色素尿的急性腎衰竭,於血液透析及適當的治療之後腎功能完全恢復的病例報告。再生不良性貧血有可能會轉變為陣發性夜間血色素尿症,並發生急性腎衰竭。有可能因為加重因子,例如:泌尿道感染,而發生急性腎衰竭。早期辨識血色素尿及迅速的處理加重因子對避免不可回復的腎衰竭是很重要的。

並列摘要


Acute renal failure (ARF) is one of the renal expressions in patients with aplastic anemia (AA)-paroxysmal nocturnal hemoglobinuria (PNH) syndrome following hemolytic crisis. We report the case of an AA patient who experienced recurrent episodes of ARF, in association with evidence of PNH. A46-year-old woman with AA was admitted because of oliguria and dark urine following a urinary tract infection (UTI) caused by Candida. PNH with ARF complication was diagnosed. Hemodialysis treatment was performed nine times and her renal function recovered. However, she suffered from recurrent hemoglobinuria and acute deterioration of renal function 4 months later. The renal biopsy showed features of acute tubular necrosis, deposition of hemosiderin and positive urate stain in the proximal tubular cells, without vascular thrombosis. The patient received management by adequate hydration, diuretics and alkalization. Her renal function recovered completely. This case report shows an AApatient experiencing recurrent episodes of hemoglobinuric ARF within a short period and achieving complete recovery of renal function after hemodialysis and suitable supportive treatment. In conclusion, AA might evolve into PNH and become complicated with presentation of severe ARF. It may occur with precipitating episodes, such as the UTI in this case. Early recognition of hemoglobinuric complications and prompt treatment for precipitating disease and ARF are important to prevent progression to an irreversible adverse renal outcome.

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