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Paroxysmal Nocturnal Haemoglobinuria Following ‘Aplastic Anaemia’

Paroxysmal Nocturnal Haemoglobinuria Following ‘Aplastic Anaemia’ 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 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Acta Haematologica Karger

Paroxysmal Nocturnal Haemoglobinuria Following ‘Aplastic Anaemia’

Acta Haematologica , Volume 38 (1): 6 – Jan 1, 2009

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Publisher
Karger
Copyright
© 1967 S. Karger AG, Basel
ISSN
0001-5792
eISSN
1421-9662
DOI
10.1159/000209000
Publisher site
See Article on Publisher Site

Abstract

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

Journal

Acta HaematologicaKarger

Published: Jan 1, 2009

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