Severe haemolysis and renal failure in a patient with paroxysmal nocturnal haemoglobinuria.

Jackson, GH; Noble, Richard; Maung, Z. T.; Main, Janice; Smith, S R; Reid, Michaela

doi:10.1136/jcp.45.2.176

Cited by 9 publications

(6 citation statements)

References 3 publications

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“…Chow et al [2] reviewed seven patients with PNH and acute renal failure (ARF) [1,[6][7][8]10]. Five of these patients were diagnosed to have PNH when they presented with ARF, as was the case with our patients.…”

Section: Discussionmentioning

confidence: 66%

“…Of these, the deficiency of decay-accelerating factor (DAF; CD55) and membrane inhibitor of reactive lysis (MIRL; CD59) renders the red blood cells particularly sensitive to lysis via the alternative pathway of complement activation. Although rare, both acute and chronic kidney involvement in PNH has been described in the literature [1][2][3][4][5][6][7][8][9][10][11]. Urinary tract infection and thrombosis of the renal vein are also known complications of PNH.…”

Section: Introductionmentioning

confidence: 99%

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Spectrum of renal involvement in paroxysmal nocturnal hemoglobinuria: report of three cases and a brief review of the literature

et al. 2008

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Renal involvement in paroxysmal nocturnal hemoglobinuria (PNH) is usually clinically not apparent but, in cases with clinical involvement, varies from reversible acute dysfunction to chronic irreversible damage. We report two cases of acute kidney injury and one case of chronic kidney disease due to PNH. In all three cases a diagnosis of PNH was made after detection of renal dysfunction. The renal involvement was documented on both histology and on imaging studies. Finally, we briefly review the main literature data on renal involvement in PNH.

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Section: Discussionmentioning

confidence: 66%

Section: Introductionmentioning

confidence: 99%

Spectrum of renal involvement in paroxysmal nocturnal hemoglobinuria: report of three cases and a brief review of the literature

et al. 2008

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“…Iron deposition can result in proximal tubular dysfunction [8,9,10]. In addition, chronic renal failure due to hemosiderosis and subsequent interstitial scarring can occur in patients with long-standing PNH [10,11,12,13,14].…”

Section: Case Discussionmentioning

confidence: 99%

“…Intravascular hemolysis in PNH can lead to two forms of renal disease: (1) a severe hemolytic episode of any cause (often in association with gastroenteritis) with massive hemoglobinuria can cause AKI probably from ATN [8,9], and (2) chronic hemolysis results in iron deposition in the kidneys in almost all patients. Iron deposition can result in proximal tubular dysfunction [8,9,10].…”

Section: Case Discussionmentioning

confidence: 99%

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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“…Even in these patients, blood transfusions should be used cautiously to avoid iron overload and hemosiderosis. All blood products should be washed to avoid the induction of a hemolytic episode by the complement-rich donor plasma (80,81). Corticosteroids and anabolic steroids have been reported to reduce or alleviate hemolysis, while anabolic steroids induce haptoglobin synthesis and decrease hemoglobinemia and hemoglobinuria (82)(83)(84)(85).…”

Section: Treatmentmentioning

confidence: 99%

Paroxysmal nocturnal hemoglobinuria: New discoveries about an old disease

Riley

1994

Clinical Laboratory Analysis

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Severe haemolysis and renal failure in a patient with paroxysmal nocturnal haemoglobinuria.

Cited by 9 publications

References 3 publications

Spectrum of renal involvement in paroxysmal nocturnal hemoglobinuria: report of three cases and a brief review of the literature

Spectrum of renal involvement in paroxysmal nocturnal hemoglobinuria: report of three cases and a brief review of the literature

Renal Involvement in Paroxysmal Nocturnal Hemoglobinuria

Paroxysmal nocturnal hemoglobinuria: New discoveries about an old disease

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