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Acta haemat. 38: 57-62 (1967) Department of Clinical Pathology, Royal Victoria Hospital, Belfast Paroxysmal Nocturnal Haemoglobinuria Following ‘Aplastic Anaemia’ J . H u n t e r and M. G. N elson Paroxysmal nocturnal haemoglobinuria (PNH ) is an un common, acquired disorder of undeterm ined aetiology character ised by chronic haemolytic anaem ia, constant haemosiderinuria and occasionally by bouts of haemoglobinuria, which are classic ally nocturnal. These manifestations are due to the appearance of a proportion o f intrinsically defective cells, the presence of which in the peripheral red cell population can be dem onstrated by undue sensitivity to lysis in vitro by acidified serum ( H am’s test) or after the further addition of throm bin ( C rosby’s test). Leucopenia and throm bocytopenia of m oderate degree are frequent findings, usually associated with a morphologically normal or hyperplastic bone marrow. Some patients with PN H have severe peripheral pancytopenia associated with bone marrow hypoplasia and this may occur during the course o f the disorder (1, 9). In other patients the period o f aplasia may precede the diagnosis of PNH (2, 3, 4, 7, 10, 11). Dacie (2) has suggested th at somatic m utation
Acta Haematologica – Karger
Published: Jan 1, 2009
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