Renal Involvement in Paroxysmal Nocturnal Hemoglobinuria

Abstract

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.

Renal involvement in paroxysmal nocturnal hemoglobinuria: an update on clinical features, pathophysiology and treatment

“…Interestingly, two of those patients presented with repeated episodes of AKI in combination with hemolytic anemia, not previously investigated. Several other cases of AKI in the PNH population have been reported in the current literature [15][16][17][18][19].…”

“…When renal biopsies are performed in PNH patients presented with kidney injury, among the typical findings are hemosiderin deposits in tubular cells, most prominent in the proximal tubules. Interestingly, no evidence of renal arterial or vein thrombosis has been presented in relevant case reports or in larger studies conducted in PNH patients treated with eculizumab [11][12][13][14][15][16][17][18]20]. However, the possibility that subclinical microvascular thrombosis could contribute to renal dysfunction cannot be excluded [21].…”

“…In cases of acute hemolysis, transfusions and glucocorticoid administrations are often necessary. In PNH patients, it is important for renal vein thrombosis to be ruled out using ultrasound scans, even though no episodes of AKI due to renal vein thrombosis have been reported so far [11][12][13][14][15][16][17][18]20]. In addition, it is recommended that acute thrombotic episodes should be managed with anticoagulation treatment and, in some cases, thrombolytic therapy.…”

Acute kidney injury in a postpartum woman with paroxysmal nocturnal hemoglobinuria: A case report and literature review

“…Table 2 summarizes the reported cases of AKI secondary to PNH. 3,4,[13][14][15][16][17][18][19][20][21][22] The presentation of PNH-related AKI is similar in patients with new-onset and recurrent PNH. Four of fifteen patients, including our case, had a history of other hematologic disorders such as aplastic anemia.…”

Diagnosis and management of PNH: Review and recommendations from a Belgian expert panel

“…As chronic intravascular haemolysis can lead to renal disease, unexplained haemolysis in addition to signs of renal failure is an indication for PNH testing as well. [16][17][18] (recommendation level C).…”