Paroxysmal Nocturnal Hemoglobinuria in Myelofibrosis
نویسندگان
چکیده
منابع مشابه
Paroxysmal nocturnal hemoglobinuria in myelofibrosis.
Ten consecutive patients with myeloysis. One patient had a positive sucrose fibrosis were examined for the following hemolysis test and low red cell ACHE. signs of PNH: Ham’s test, the sucrose The remaining patients had no signs of hemolysis test, low red cell acetylcholinPNH. In two of the patients, the PNH esterase (ACHE) activity and intrasigns disappeared after splenectomy. The vascular hem...
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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 کاملParoxysmal nocturnal hemoglobinuria.
Paroxysmal Nocturnal Hemoglobinuria (PNH) literally means to have episodes of hemoglobin in the urine during the night. It is a Coomb's negative rare hemolytic disorder characterized by non-malignant clonal expansion of haemopoietic stem cells due to acquired genetic mutations. A 30 years old male patient presented with 5 years history of transfusion dependent anemia with intermittent episodes ...
متن کاملParoxysmal nocturnal hemoglobinuria.
Keywords Disease name and synonym Definition and Differential Diagnosis Etiology Clinical Description Diagnostic Methods Epidemiology Management References Abstract Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disorder of the bone marrow characterized by the lack (total or partial) of all proteins normally attached to the cell membrane by the glycosylphosphatidylinositol (GPI...
متن کاملParoxysmal nocturnal hemoglobinuria.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare bone marrow failure disorder that manifests with hemolytic anemia, thrombosis, and peripheral blood cytopenias. The absence of two glycosylphosphatidylinositol (GPI)-anchored proteins, CD55 and CD59, leads to uncontrolled complement activation that accounts for hemolysis and other PNH manifestations. GPI anchor protein deficiency is almost alw...
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ژورنال
عنوان ژورنال: Blood
سال: 1970
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood.v36.4.428.428