Acute tubular necrosis in a patient with paroxysmal nocturnal hemoglobinuria

Abstract

Acute renal failure (ARF) is a well-recognized complication of paroxysmal nocturnal hemoglobinuria (PNH). The predominant mechanism is intravascular hemolysis resulting in massive hemoglobinuria ARF. We report a case of acute tubular necrosis (ATN) developed in the absence of overwhelming evidence of intravascular hemolysis in a 21-year-old man with anemia, who was eventually diagnosed to have PNH. The patient presented with rapidly deteriorating renal functions in the background of iron deficiency anemia, which was attributed to reflux esophagitis. There was no clinical or laboratory evidence of intravascular hemolysis. Renal biopsy revealed ATN with deposition of hemosiderin in the proximal tubular epithelial cells. Diagnosis of PNH was confirmed with a positive Ham's test and flow cytometry. Our case emphasizes the need to consider ATN as a possible cause for ARF in patients suspected to have PNH even in the absence of overwhelming evidence of intravascular hemolysis.

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

“…An acute hemolytic episode associated with massive hemoglobinuria may cause an episode of acute renal failure. The mechanisms behind this phenomenon include the following: (a) hypovolemia and renal ischemia; (b) direct toxicity of free hemoglobin in the tubular cells; (c) glomerular deposition of fibrin and (d) tubular obstruction by uric acid crystals and hemoglobin urinary casts-cylinders (the latter are products of heme reaction with Tamm-Horsfall protein (THP)a secretory product of the cells of the distal convoluted tubules) [29].…”

Reoperation after mitral valve repair in viewpoints of kidney injury as well as hemolytic anemia

“…In addition, the anticoagulation therapy was needed for the patient because intravascular hemolysis and insufficient endothelialization of the annuloplasty ring were considered to be risk of thrombosis. Chronic hemolysis and subsequent proximal tubular injury resulting from iron deposition could cause progression to CKD in cases of paroxysmal nocturnal hemoglobinuria (PNH), which is representative of intravascular hemolysis [28]. However, kidney injury in cases of PNH can progress even without clinical evidence of intravascular hemolysis [28].…”

“…Chronic hemolysis and subsequent proximal tubular injury resulting from iron deposition could cause progression to CKD in cases of paroxysmal nocturnal hemoglobinuria (PNH), which is representative of intravascular hemolysis [28]. However, kidney injury in cases of PNH can progress even without clinical evidence of intravascular hemolysis [28]. Therefore, the possibility that chronic hemolysis can result in renal iron deposition and subsequent progression to CKD must be considered, even without clinical evidence of anemia.…”

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.…”

“…Table summarizes the reported cases of AKI secondary to PNH . The presentation of PNH‐related AKI is similar in patients with new‐onset and recurrent PNH.…”