Paroxysmal nocturnal hemoglobinuria treated with ACTH without benefit.
نویسندگان
چکیده
منابع مشابه
Intestinal perforation in a patient with paroxysmal nocturnal hemoglobinuria
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder that manifests with hemolytic anemia, thrombosis, and peripheral blood cytopenias.Acute abdominal pain is one of the PNH clinical manifestations due to venous thrombosis of intra-abdominal sites including hepatic, portal, mesenteric, and splenic veins.Eculizumaband allogeneic bone marrow transplantation (BMT) arethe only w...
متن کاملTreatment of paroxysmal nocturnal hemoglobinuria with ACTH.
pAROXYSMAL NOCTURNAL HEMOGLOBINURIA is a rare hemolytic disease characterized by intravascular hemolysis and excretion of hemoglobin in the urine during sleep. Crosby,’ in an extensive review of the literature, found reports of 123 cases of this condition and gave credit to StrUbing for first recognizing it as an entity in 1882. It was not until another description was published by Marchiafava2...
متن کاملParoxysmal nocturnal hemoglobinuria clones in severe aplastic anemia patients treated with horse anti-thymocyte globulin plus cyclosporine.
BACKGROUND Clones of glycosylphosphatidylinositol-anchor protein-deficient cells are characteristic in paroxysmal nocturnal hemoglobinuria and are present in about 40-50% of patients with severe aplastic anemia. Flow cytometry has allowed for sensitive and precise measurement of glycosylphosphatidylinositol-anchor protein-deficient red blood cells and neutrophils in severe aplastic anemia. DE...
متن کاملA patient with paroxysmal nocturnal hemoglobinuria being treated with eculizumab who underwent laparoscopic cholecystectomy: report of a case
Paroxysmal nocturnal hemoglobinuria (PNH) is acquired hemolytic anemia characterized by symptoms such as anemia and hemoglobinuria. In recent years, eculizumab as an anti-complement (C5) monoclonal antibody has been used for PNH and shown to have marked effects. We performed laparoscopic cholecystectomy in a patient with PNH being treated with eculizumab, and could avoid the risk of perioperati...
متن کاملAllogeneic stem cell transplantation in paroxysmal nocturnal hemoglobinuria.
BACKGROUND In the era of eculizumab, identifying patients with paroxysmal nocturnal hemoglobinuria who may benefit from allogeneic stem cell transplantation is challenging. DESIGN AND METHODS We describe the characteristics and overall survival of 211 patients transplanted for paroxysmal nocturnal hemoglobinuria in 83 EBMT centers from 1978 to 2007. Next, we conducted a comparison with a coho...
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ورودعنوان ژورنال:
- Blood
دوره 7 10 شماره
صفحات -
تاریخ انتشار 1952