Renal manifestations in paroxysmal nocturnal hemoglobinuria

Abstract

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.

Clinical profile and outcome of pigment-induced nephropathy

“…In a study of 14 patients of PNH by Ram et al ., AKI was noted in six (42.8%) patients, five of whom had HD [ 27 ]. Renal biopsy was done in four patients and all showed prominent hemosiderin pigments and acute tubular necrosis.…”

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].…”

A Rare Case of Paroxysmal Nocturnal Hemoglobinuria With Bilateral Renal Vein Thrombosis

“…Less commonly, patients with PNH can also present with manifestations of smooth muscle dystonia such as abdominal pain, dysphagia and erectile dysfunction and are at a six times increased risk of chronic kidney disease, with multiple mechanisms, including tubular atrophy, interstitial fibrosis and microinfarcts playing a role [ 1 , 6 ]. Our patient presented with abdominal pain, which could, however, also be attributed to extensive intra-abdominal thrombosis.…”

“…Renal manifestations in PNH are not uncommon. A retrospective analysis of 14 patients at a single setup between 1998 and 2004 revealed acute kidney injury (AKI) in six (42.8%), Fanconi syndrome in three (21.4%) and unilateral renal vein thrombosis in two (14.2%) patients [ 6 ]. In 2012, a case of PNH with renal vein infarction was also reported [ 7 ].…”