PAROXYSMAL NOCTURNAL HAEMOGLOBINURIA

Publisher Website
Google Scholar
Abstract
Paroxysmal nocturnal haemoglobinuria (PNH) is a relatively rare and fascinating haemolytic anaemia of uncertain aetiology. Abnormal populations of red ceils, white cells and platelets which differ in a number of ways from normal are presumed to arise from a clone of stem cells, altered through injury or Insult at present unrecognized. Several different forms of clinical presentation are recognized, and the disease is characterized by exacerbations and remissions. Treatment is symptomatic, and contention surrounds the use of many suggested therapeutic agents. PNH should enter the differential diagnosis of any patient presenting with a puzzling anaemia (particularly if It is haemolytic), pancytopenia, unexplained thrombosis or a history of unexplained transfusion reaction, since a precise diagnosis will Influence further management.