Acute renal failure in a patient with severe hemolysis
Tarih
2007Yazar
Ure, Umit
Ozbay, Gulsen
Karaali, Zeynep
Selcuk, Dogan
Kazancioglu, Rumeyza
Kirkizlar, Onur
Kendir, Mehmet
Üst veri
Tüm öğe kaydını gösterÖzet
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 Phosphatidylinositolglycan 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.
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- Makale [92797]