Acute renal failure in a patient with severe hemolysis
International Urology and Nephrology, cilt.39, sa.2, ss.651-654, 2007 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 39 Sayı: 2
- Basım Tarihi: 2007
- Doi Numarası: 10.1007/s11255-006-9096-3
- Dergi Adı: International Urology and Nephrology
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
- Sayfa Sayıları: ss.651-654
- Anahtar Kelimeler: Acute renal failure, Hematuria, Hemolysis, Paroxysmal nocturnal hemoglobinuria
- Trakya Üniversitesi Adresli: Hayır
Özet
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. © Springer Science+Business Media B.V. 2007.