title: 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.
authors.affiliation: Internal Medicine Clinic, Haseki Education and Research Hospital, Adivar street, Aksaray/Fatih, Istanbul, Turkey. onurkirkizlar@gmail.com
authors.forename: Onur
authors.initials: O
authors.lastname: Kirkizlar
authors.forename: Mehmet
authors.initials: M
authors.lastname: Kendir
authors.forename: Zeynep
authors.initials: Z
authors.lastname: Karaali
authors.forename: Umit
authors.initials: U
authors.lastname: Ure
authors.forename: Gulsen
authors.initials: G
authors.lastname: Ozbay
authors.forename: Dogan
authors.initials: D
authors.lastname: Selcuk
authors.forename: Rumeyza
authors.initials: R
authors.lastname: Kazancioglu
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology*; D000328:Adult; D005260:Female; D006461:Hemolysis; D006801:Humans; D012720:Severity of Illness Index