Reversible Acute Kidney Injury Caused by Paroxysmal Nocturnal Hemoglobinuria

Qi, Ka; Zhang, Xueguang; Liu, Shuwen; Zhong, Yin; Chen, Xiangmei; Wu, Di

doi:10.1097/maj.0b013e3181f515b9

Cited by 16 publications

(20 citation statements)

References 12 publications

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“…There is evidence reporting that patients with PNH and less advanced stages of AKI can be managed successfully without hemodialysis [15]. There is a case report of a 57-year-old male who presented with diarrhea, darkened urine, jaundice and increased blood urea nitrogen and creatinine, whose renal biopsy indicated ATN and hemosiderin deposition.…”

Section: Case Discussionmentioning

confidence: 99%

“…A CD55 and CD59 assay, Ham test and sugar-water hemolysis test confirmed the diagnosis of PNH. He was managed conservatively with fluids, diuresis and urine alkalization and gradually regained near-normal renal function [15], but was oliguric with advanced AKI and required urgent hemodialysis. Moreover, the renal pathology sample showed the most affected area of the kidney (10 out of 12 glomeruli sclerosed) which misrepresented the whole kidney histology as his AKI resolved showing that he probably had much more viable renal tissue.…”

Section: Case Discussionmentioning

confidence: 99%

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Renal Involvement in Paroxysmal Nocturnal Hemoglobinuria

2013

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Renal involvement in paroxysmal nocturnal hemoglobinuria (PNH) is not usually apparent but in cases with clinical involvement varies from reversible acute dysfunction to chronic irreversible damage. Early diagnosis and treatment is crucial to prevent disease progression and irreversible chronic kidney disease (CKD). The ultimate outcome of CKD in many patients is the need for renal replacement therapy, which necessitates ever-growing dialysis and transplantation programs, thereby imposing a significant economic burden on the healthcare system. In a third-world country like Pakistan, increased burden due to CKD can be very hard on families. Doctor visits, hospitalization, and dialysis are all out-of-pocket expenses, therefore prevention, early detection, and timely intervention are the only cost-effective strategies. We report a case of acute kidney injury (AKI) due to PNH. This case shows AKI as one of the complications of PNH which may have a clinical course like acute tubular necrosis (ATN). This could be due to ATN or AKI superimposed on CKD due to hemosiderin deposits in the renal tubular epithelial cells. Our patient was dialyzed initially and discharged with a permanent catheter in place with advice to continue dialysis three times a week. He required dialysis for 1 week then started producing urine. His subsequent outpatient visits showed improved renal function. The permanent catheter was removed and maintenance dialysis was stopped. Here, we briefly review the literature on renal involvement in PNH, treatment options for PNH and pigment-induced nephropathy followed by a question-and-answer session at the clinicopathological conference held on March 4, 2011, at Sindh Institute of Urology and Transplantation in Karachi, Pakistan.

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Section: Case Discussionmentioning

confidence: 99%

Section: Case Discussionmentioning

confidence: 99%

Renal Involvement in Paroxysmal Nocturnal Hemoglobinuria

2013

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“…Yet patients of PNH have been found to have both acute and chronic renal insufficiency unattributable to other diseases. [234567] All the three patients in our study presented primarily with renal complaints and were suspected as PNH on subsequent evaluation of urine sediment and renal biopsy. Renal biopsy findings in these patients had a wide spectrum of findings.…”

Section: Discussionmentioning

confidence: 95%

“…[1] Although rare, both acute and chronic kidney involvement can occur in PNH. [234567] Renal failure as the primary presentation leading to subsequent diagnosis of PNH is even rarer. [567] We received 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.…”

Section: Introductionmentioning

confidence: 99%

Renal biopsy in paroxysmal nocturnal hemoglobinuria: An insight into the spectrum of morphologic changes

2017

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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 per se 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.

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“…Under normal circumstances, hemosiderin deposited is mildly toxic but in the presence of low circulating blood volume and aciduria it can induce acute renal failure. 9 Our patient presented with high grade fever together with less oral fluid intake leading to reduction in circulating fluid volume thus predisposing hemosiderin induced acute renal failure. We managed her renal dysfunction by increasing circulating fluid volume, use of diuretics and together with steroid to reduce complement mediated hemolysis.…”

Section: Discussionmentioning

confidence: 99%