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

Nair, Rathish; Khaira, Ambar; Sharma, Alok; Mahajan, Sandeep; Dinda, Amit Kumar

doi:10.1007/s11255-008-9356-5

Cited by 39 publications

(41 citation statements)

References 13 publications

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“…In all these cases a diagnosis of PNH was made after detection of renal dysfunction [16]. There is a case report from India in which a 70-year-old male had had chronic idiopathic thrombocytopenic purpura since 1990 and who presented with low hemoglobin (Hb).…”

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%

“…Hb gradually rose to 8 g/dl without any transfusions. The patient continued a regular follow-up and maintained normal renal parameters [16]. In another case, a 49-year-old male from India with the diagnosis of aplastic anemia was found to have had dark-colored urine for 10 months.…”

Section: Case Discussionmentioning

confidence: 99%

Renal Involvement in Paroxysmal Nocturnal Hemoglobinuria

2013

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“…25 Three cases of paroxysmal nocturnal hemoglobinuria with renal hemosiderosis and renal failure were reported by Nair and colleagues. 10 Most patients with renal hemosiderosis also have renal failure.…”

Section: Discussionmentioning

confidence: 99%

“…This disorder is a disease, like sickle cell anemia and thalassemia, in which chronic blood loss requires frequent blood transfusions (though beta minor thalassemia has been associated with hemosiderin deposits in the liver in patients with non-alcoholic fatty liver disease independent of any transfusions). 7,8 Also, renal hemosiderosis is a complication of chronic intravascular hemolytic states, such as hemolytic anemia, paroxysmal nocturnal hemoglobinuria (PNH) and mechanical hemolysis after inserting a prosthetic cardiac valve 9,10 or black-water fever as well. 3 Renal hemosiderosis (blue kidney) is the anatomic indicator of severe intravascular hemolysis.…”

Section: Introductionmentioning

confidence: 99%

Normal black kidney

Yarmohamadi

Rezayat

Memar

et al. 2014

CUAJ

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A black kidney has 3 major differential diagnoses: hemosiderosis, lipofuscin pigment and melanotic renal cell carcinoma. Excluding lipofuscin, the other 2 are accompanied by an abnormal renal function. We report on a 25-year-old man who intended to donate a kidney to his cousin. On the operating room table when we incised the left flank region and exposed the kidney, we found a firm and black kidney so the operation was cancelled due to potential vascular injuries. Days after the incomplete procedure, we reviewed the donor's biochemistry and imaging to reassess his renal function, but the results showed quite normal renal function again. The result of Ham test was also negative. Two weeks later, we began the operation, removed the same left kidney and found that it was in the same conditions as it was before. We took the opportunity to send needle biopsies of the kidney for histopathologic analysis. The analysis showed a melanotic kidney without pathological changes in glomeruli and interstitium and vessels. A black kidney may result in hemosiderin, lipofuscin or melanin deposits in the kidney, which can confirm the diagnosis; however, special tests for underlying disease and renal function should be considered. Some causes of black kidney lead to abnormal function, but our patients's kidney returned to normal.

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“…Both acute and chronic renal failure have been described in PNH patients (Nair et al 2008). Acute renal insufficiency is usually seen in concomitance with hemolytic crisis, as a result of massive hemoglobinuria.…”

Section: Other Clinical Manifestationsmentioning

confidence: 99%

Paroxysmal Nocturnal Hemoglobinuria

Rotoli

Luzzatto

Principles of Molecular Medicine

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

Cited by 39 publications

References 13 publications

Renal Involvement in Paroxysmal Nocturnal Hemoglobinuria

Renal Involvement in Paroxysmal Nocturnal Hemoglobinuria

Normal black kidney

Paroxysmal Nocturnal Hemoglobinuria

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