A blue kidney  chronic renal failure as a consequence of siderosis in paroxysmal nocturnal hemoglobinuria?

Kümpers, Philipp; Herrmann, Amanda; Lotz, Jeffrey C.; Mengel, M.; Schwarz, Albin

doi:10.5414/cnp66210

Cited by 10 publications

(9 citation statements)

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“…[726] There are a few case reports of CKD in PNH; but in majority of them, the diagnosis of CKD was not clear cut. [27] In a report, a 74-year-old male with PNH developed acute on CKD after an episode of intercurrent urinary tract infection and subsequent hemolytic crisis. The decline in GFR was believed to be secondary to mild chronic hemolysis.…”

Section: Discussionmentioning

confidence: 99%

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Renal manifestations in paroxysmal nocturnal hemoglobinuria

et al. 2017

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Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired chronic disorder characterized by a triad of clinical features - hemolytic anemia, pancytopenia, and thrombosis. Not many reports of renal involvement in PNH are available in literature. We present a case series of PNH with renal involvement. We present the data of PNH patients who attended to Departments of General Medicine and Nephrology at a government-run tertiary care institute in South India. The diagnosis of PNH in these patients during initial phase, between 1998 and 2004 was based on sucrose lysis and Ham's test. After 2004, the diagnosis was based on flow cytometry to detect CD59 (membrane inhibitor of reactive lysis), a glycoprotein, and CD55 (decay accelerating factor) in regulation of complement action. The patient data were collected from 1998 to 2014. There were 14 patients of PNH in this period. The mean age was 37 years and the range was 16–68 years. There were eight females. Acute kidney injury (AKI) was noted in six patients. Dialysis was performed in four of them. The mean serum creatinine and urea at the initiation of dialysis were 5.4 ± 0.6 and 64.1 ± 6.1 mg/dl, respectively. The median number of hemodialysis sessions done was four. Renal biopsy was done in four patients. In three patients, the urinalysis and serum chemistry were suggestive of Fanconi syndrome. In our patients, three renal manifestations of PNH were identified. They were AKI, renal vessel thrombosis, and Fanconi syndrome. Chronic renal failure was not identified.

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Section: Discussionmentioning

confidence: 99%

“…In retrospect, these findings were explained by a 15-year history of hypertension and a 4-year medication with cyclosporine he used. [27] Another 70-year-old male with a diagnosis of chronic idiopathic thrombocytopenic purpura had presented with low hemoglobin. He was found to have PNH.…”

Section: Discussionmentioning

confidence: 99%

Renal manifestations in paroxysmal nocturnal hemoglobinuria

et al. 2017

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“…[9] However, Kümpers et al . [15] have raised questions on attributing these morphologic changes to siderosis in PNH, thereby implicating other factors such as chronic hypertension in the pathogenesis. We opine that intrarenal hypertension itself could be a manifestation of hemoglobin toxicity due to PNH.…”

Section: Discussionmentioning

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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“…Nevertheless, PH and CKD are not commonly described in PNH patients, and are not included among the common causes of death in the largest epidemiologic studies [40][41][42]. PH was observed in a single study only [58], while most reports dealing with renal function refer to acute renal failure secondary to paroxysms of hemolysis and hemaglobinuria [44,59,60]. Therefore, the real clinical impact of these finding has to be assessed in appropriate studies, and PNH should not be necessarily considered a progressive disease constantly leading to endstage organ damage.…”

Section: Eculizumab and Pnh: Any Additional Benefit?mentioning

confidence: 98%

Achievements and Limitations of Complement Inhibition by Eculizumab in Paroxysmal Nocturnal Hemoglobinuria: The Role of Complement Component 3

Risitano¹,

Perna²,

Selleri

2011

MRMC

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Paroxysmal nocturnal hemoglobinuria (PNH) is a hematological disorder characterized by complementmediated hemolytic anemia, thrombophilia and bone marrow failure. The clinical hallmark of PNH is evident chronic hemolysis due to the absence of the complement regulators CD55 and CD59 on PNH erythrocytes. Intravascular hemolysis drives the major clinical features of PNH, including anemia, hemoglobinuria, fatigue and other hemolysisrelated disabling symptoms, such as painful abdominal crises, dysphagia and erectile dysfunction. A peculiar thromboembolic risk has been associated with the hemolysis in PNH, but its pathophysiologic cause remains unclear. The treatment of PNH has remained supportive until a few years ago, when the first complement inhibitor, designated eculizumab, became available. Chronic treatment with eculizumab results in sustained control of intravascular hemolysis, leading to hemoglobin stabilization and transfusion independence in half of the patients. However, residual anemia may persist in a substantial fraction of patients. Recent observations by different groups, including our own, have demonstrated that residual hemolysis may be due to persistent activation of the early phases of the complement cascade, leading to progressive C3-deposition on PNH erythrocytes and possible subsequent extravascular hemolysis through the reticuloendothelial system. Here we critically review the available clinical results of eculizumab treatment for PNH patients, pointing out the recent insights into the pathophysiology of the disease. We discuss the role of the different components of the complement cascade leading to hemolysis, in both the absence and presence of the terminal effector pathway inhibition by eculizumab. Finally, we provide a theoretical rationale for the development of novel strategies of complement inhibition which could in the future further improve on the already substantial efficacy of eculizumab.

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A blue kidney chronic renal failure as a consequence of siderosis in paroxysmal nocturnal hemoglobinuria?

Cited by 10 publications

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Renal manifestations in paroxysmal nocturnal hemoglobinuria

Renal manifestations in paroxysmal nocturnal hemoglobinuria

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

Achievements and Limitations of Complement Inhibition by Eculizumab in Paroxysmal Nocturnal Hemoglobinuria: The Role of Complement Component 3

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A blue kidney  chronic renal failure as a consequence of siderosis in paroxysmal nocturnal hemoglobinuria?

Cited by 10 publications

References 0 publications

Renal manifestations in paroxysmal nocturnal hemoglobinuria

Renal manifestations in paroxysmal nocturnal hemoglobinuria

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

Achievements and Limitations of Complement Inhibition by Eculizumab in Paroxysmal Nocturnal Hemoglobinuria: The Role of Complement Component 3

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A blue kidney chronic renal failure as a consequence of siderosis in paroxysmal nocturnal hemoglobinuria?