Biochemical background of paroxysmal nocturnal hemoglobinuria
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چکیده
منابع مشابه
Paroxysmal 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...
متن کاملParoxysmal Nocturnal Hemoglobinuria
Paroxysmal nocturnal hemoglobinuria (PNH) is a complex hematological disorder resulting in a quite unique clinical syndrome. In fact, the typical clinical presentation encompasses three distinct hematological manifestations, i.e., hemolytic anemia, bone marrow failure and thrombophilia (Dunn et al 2000; Parker & Ware 2003; Notaro & Luzzatto 2003). Thus, the term PNH covers only one feature of t...
متن کاملTreatment of paroxysmal nocturnal hemoglobinuria.
Patients with PNH may be treated with a number of known agents. As in all patients with a chronic disease, a regimen tolerable over a long period of time must be selected. Knowledge and anticipation of complications and their proper treatment are essential parts in the treatment. When these principals are used, many patients may live reasonable lives for very long periods of time.
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ژورنال
عنوان ژورنال: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
سال: 1999
ISSN: 0925-4439
DOI: 10.1016/s0925-4439(99)00068-x