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International Journal of Hematology
Published in: International Journal of Hematology 2/2010

01-03-2010 | Images in Hematology

Paroxysmal nocturnal hemoglobinuria turning Coombs-positive

Authors: Alexander Röth, Sven Peine, Ulrich Dührsen

Published in: International Journal of Hematology | Issue 2/2010

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Excerpt

Paroxysmal nocturnal hemoglobinuria (PNH) represents a Coombs-negative hemolytic anemia. Due to mutations of the PIG-A gene, the affected progeny cells are deficient in all GPI-anchored surface proteins, including the complement regulators CD55 and CD59. Therefore, PNH RBCs are highly vulnerable to activated complement with the formation of the membrane attack complex (MAC) resulting in chronic intravascular hemolysis as the underlying cause of morbidities and mortality in PNH. …
Literature
1.
Risitano AM, Marando L, Seneca E, Rotoli B. Hemoglobin normalization after splenectomy in a paroxysmal nocturnal hemoglobinuria patient treated by eculizumab. Blood. 2008;112:449–51.CrossRefPubMed
Metadata
Title
Paroxysmal nocturnal hemoglobinuria turning Coombs-positive
Authors
Alexander Röth
Sven Peine
Ulrich Dührsen
Publication date
01-03-2010
Publisher
Springer Japan
Published in
International Journal of Hematology / Issue 2/2010
Print ISSN: 0925-5710
Electronic ISSN: 1865-3774
DOI
https://doi.org/10.1007/s12185-010-0493-0

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