Acute renal failure in a patient with severe hemolysis

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal disorder of bone marrow. It is characterized by blood cells lacking membrane proteins that are normally attached by the glycosylphosphatidylinositol (GPI) anchor. The cellular defect arises in a hematopoetic stem cell and is due to somatic mutation of the Phosphatidylinositol-glycan protein-A gene (PIG-A gene), encoding a protein needed for the biosynthesis of the anchor GPI. Paroxysmal nocturnal hemoglobinuria is presented by intravascular hemolysis, cytopenias, frequent infections, bone marrow hypoplasia, and a high incidence of life threatening venous thrombosis. Kidney involvement is usually benign and secondary to chronic tubular deposition of hemosiderin. Acute renal failure may occur in association with a hemolytic crisis. Here we report a case of 40-year-old woman with hematuria, pancytopenia, and acute renal failure due to PNH.

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

“…Among them, three patients subsequently developed CKD (chronic glomerular filtration rate (GFR) < 60 ml/minute/1.73 m 2 ). Similar studies have reported AKI as the first clinical sign in PNH in a total of five patients [11][12][13][14]. Interestingly, two of those patients presented with repeated episodes of AKI in combination with hemolytic anemia, not previously investigated.…”

“…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

“…11 To our knowledge, two reports indicated that MRI was helpful in confirming the diagnosis of AKI secondary to PNH. 3,12 Features typical of renal cortical hemosiderosis were found in our patient, suggesting that her kidney injury was, at least in part, caused by PNH. Table 2 summarizes the reported cases of AKI secondary to PNH.…”

“…As a noninvasive alternative examination, MRI provides evidence of the renal cortical hemosiderosis characteristic of PNH, including reversed renal cortex‐medulla differentiation on T1 weighted images and a substantial loss of cortical signal intensity on both T1 and T2 weighted images . To our knowledge, two reports indicated that MRI was helpful in confirming the diagnosis of AKI secondary to PNH . Features typical of renal cortical hemosiderosis were found in our patient, suggesting that her kidney injury was, at least in part, caused by PNH.…”