Acute kidney injury could be sign of PNH, case report finds
Treatment helped to prevent progression in man, 19, in India
In rare cases, acute kidney injury may be sign of paroxysmal nocturnal hemoglobinuria (PNH), as described in a recent case report.
“Although renal dysfunction in PNH is rare and as renal dysfunction is a dreadful complication, timely intervention is life-saving for the patient who requires hemodialysis,” the scientists wrote.
The report, “Paroxysmal nocturnal hemoglobinuria presenting as acute renal injury,” was published in the International Journal of Research in Medical Sciences.
PNH symptoms are common to various diseases, complicating a diagnosis
PNH is characterized by the premature breakdown of blood cells. Some disease symptoms, such as fatigue, dark urine, and abdominal pain, also are common in other conditions, which can make diagnosing PNH more challenging.
Researchers in India described the case of a young man with PNH that manifested in the form of acute kidney injury.
The 19-year-old had dark colored urine for the past two years, and a yellowish discoloration in the skin and eyes for about a month.
A bone marrow analysis confirmed the presence megaloblastic anemia and myelodysplastic syndrome (MDS). Megaloblastic anemia is a type of anemia characterized by the presence of abnormally large red blood cells that fail to work properly; MDS is a rare type of blood cancer that’s marked by the presence of immature blood cells.
The patient was treated for six months with danazol, a steroid, and the immunosuppressant cyclosporine, and a follow-up bone marrow biopsy showed improvements. He received a blood transfusion every month.
He returned to the hospital due to fatigue that had persisted for a month and three episodes of hematuria (blood in the urine). These symptoms were accompanied by nausea, vomiting, and a decrease in urine output.
Blood work showed low levels of hemoglobin, the protein that carries oxygen in red blood cells, as well as low white blood cell and platelet counts. Urea and creatinine levels were markedly elevated, indicating poor kidney function.
Treatment ranged from danazol, a steroid, to hemodialysis
The patient started hemodialysis, a type of treatment in which blood goes through a filter, called a dialyzer, that acts as an artificial kidney, removing extra water and waste from the blood. In total, he completed five sessions.
An ultrasound showed his liver and kidneys were normal size. Blood tests for infections, including dengue fever, malaria, and scrub typhus, came out negative. A urine test revealed the presence of hemosiderin, consistent with excess red blood cell destruction, or hemolysis. The patient was ultimately diagnosed with PNH-hypoplastic MDS. Of note, hypoplastic MDS is a myelodysplastic syndrome subtype marked by the presence of a low number of cells in the bone marrow.
He received treatment with danazol and an oral steroid and was advised to undergo a stem cell transplant. During his hospital stay, his urine volume gradually increased and he stopped hemodialysis. He was discharged after blood tests showed a marked decrease in creatinine levels.
“This case shows that PNH may present with [acute kidney injury] when hemolysis occurs and hemosiderin deposits in the [kidney],” the researchers wrote. “Early diagnosis and treatment are crucial to prevent disease progression.”
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