Immunophenotypic analysis of reticulocytes in paroxysmal nocturnal hemoglobinuria
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چکیده
منابع مشابه
Immunophenotypic analysis of reticulocytes in paroxysmal nocturnal hemoglobinuria.
The hematologic disorder paroxysmal nocturnal hemoglobinuria (PNH) occurs following an acquired somatic mutation in the Piga gene within a bone marrow stem cell. The progeny of this mutated cell cannot synthesize glycosylphosphatidylinositol (GPI) anchors, with a resultant deficiency in surface expression of all GPI-linked proteins. The protean clinical manifestations of PNH presumably result f...
متن کاملLife Span of Reticulocytes in Paroxysmal Nocturnal Hemoglobinuria.
P REVIOUS OBSERVATIONS have demonstrated the shortened life span of the red cell in patients with paroxysmal nocturnal hemoglohinuria ( PNH ).1.2 Studies of the survival of homologous PNH red cells by the Ashby technic and of autologous PNH cells labeled with radioactive sodium chromate have demonstrated a short and biphasic red cell survival curve in the patient and normal recipient. This life...
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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...
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ژورنال
عنوان ژورنال: Blood
سال: 1995
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood.v86.4.1586.bloodjournal8641586