منابع مشابه
Paroxysmal Nocturnal Haemoglobinuria
A Clinico-Pathological Conference held in the University of Bristol on the 6th November 1962 chairman: professor t. f. hewer Dr. Crow: This lady was 57 at the time of death and although her early history isi little scanty it seems likely that the disease which ultimately caused her death first presented around the age of 21. We have nothing like complete notes up to 1953, but even so her notes ...
متن کاملEculizumab for paroxysmal nocturnal haemoglobinuria.
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...
متن کاملParoxysmal nocturnal haemoglobinuria: nature's gene therapy?
The development of paroxysmal nocturnal haemoglobinuria (PNH) requires two coincident factors: somatic mutation of the PIG-A gene in one or more haemopoietic stem cells and an abnormal, hypoplastic bone marrow environment. When both of these conditions are met, the fledgling PNH clone may flourish. This review will discuss the pathophysiology of this disease, which has recently been elucidated ...
متن کاملParoxysmal 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.
متن کاملParoxysmal nocturnal haemoglobinuria and diabetes mellitus.
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: Medical Journal of Australia
سال: 1974
ISSN: 0025-729X,1326-5377
DOI: 10.5694/j.1326-5377.1974.tb71131.x