Paroxysmal nocturnal haemoglobinuria and Budd-Chiari syndrome.
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منابع مشابه
Paroxysmal nocturnal haemoglobinuria and Budd-Chiari syndrome.
An 11 year old boy developed pancytopenia, haemolysis, and Budd-Chiari syndrome. The venous thrombosis extended to involve other intra-abdominal vessels before paroxysmal nocturnal haemoglobinuria was recognised as the underlying haematological abnormality. Earlier diagnosis would have made curative bone marrow transplantation a possibility.
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The complement system plays a central part in both innate and acquired immunity, but the contribution of complement activation to pathobiology is largely ancillary. An exception to the non-dominant role of complement in disease is the haemolytic anaemia of paroxysmal nocturnal haemoglobinuria (PNH). The intravascular haemolysis that is the clinical hallmark of PNH is a consequence of deficiency...
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Paroxysmal nocturnal hemoglobinuria (PNH) is a progressive, life-threatening disorder characterized by chronic intravascular hemolysis caused by uncontrolled complement activation. Hepatic vein thrombosis (Budd-Chiari syndrome) is common in PNH patients. This case report describes the response to eculizumab (a humanized monoclonal antibody that inhibits terminal complement activation) in a 25-y...
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Paroxysmal nocturnal haemoglobinura is an acquired disorder of the red cell membrane rendering the cell especially liable to lysis by activated complement. There may be a single or several clones of sensitive red cells and the disorder represents a defect at the pluripotential stem cell stage. This is supported by the findings of leucopenia and thrombocytopenia and the possible progression to p...
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ژورنال
عنوان ژورنال: Archives of Disease in Childhood
سال: 1995
ISSN: 0003-9888,1468-2044
DOI: 10.1136/adc.72.3.241