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01.06.2011 | ORIGINAL ARTICLE

The Patterns of MHC Association in Aplastic and Non-aplastic Paroxysmal Nocturnal Hemoglobinuria

verfasst von: Jacek Nowak, Renata Mika-Witkowska, Ewa Mendek-Czajkowska, Marta Rogatko-Koroś, Elżbieta Graczyk-Pol, Hanna Pyl, Aneta Klimczak, Małgorzata Wójcik, Monika Prochorec-Sobieszek, Renata Maryniak, Barbara Żupańska

Erschienen in: Archivum Immunologiae et Therapiae Experimentalis | Ausgabe 3/2011

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Abstract

The deficiency of glycosyl-phosphatidylinositol (GPI)-anchored proteins in plasma membranes of PIG-A gene mutated hematopoietic stem cells (HSCs) is so far insufficient to explain the domination of paroxysmal nocturnal hemoglobinuria (PNH) clone over the normal HSC. We attempted to elucidate possible link between MHC and initial severe aplastic anemia (ISAA/PNH) type and non-aplastic (n/PNH) outcome of PNH. In 50 PNH patients assigned as ISAA/PNH (n = 13), n/PNH (n = 33) or nonassigned (n = 4) and 200 ethnically matched controls we analyzed MHC associations. Our data confirmed strong associations of DRB1*15:01 (RR = 3.51, p = 0.0011) and DQB1*06:02 (RR = 7.09, p = 0.000026) alleles, especially with n/PNH subtype. B*18:01 allele was associated with increased risk of ISAA/PNH subtype (RR = 5.25, p = 0.0028). We conclude that both class II and class I MHC alleles are associated with different subsets of PNH. Clonal selection of PIG-A mutated cells with cognate metabolic block is associated with MHC class II alleles DRB1*15:01 and DQB1*06:02 independent from initial severe AA clone selection. MHC class I molecule B*18:01 can additionally influence the domination of PNH clone in PNH subjects with initial severe aplastic anemia.
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Metadaten
Titel
The Patterns of MHC Association in Aplastic and Non-aplastic Paroxysmal Nocturnal Hemoglobinuria
verfasst von
Jacek Nowak
Renata Mika-Witkowska
Ewa Mendek-Czajkowska
Marta Rogatko-Koroś
Elżbieta Graczyk-Pol
Hanna Pyl
Aneta Klimczak
Małgorzata Wójcik
Monika Prochorec-Sobieszek
Renata Maryniak
Barbara Żupańska
Publikationsdatum
01.06.2011
Verlag
SP Birkhäuser Verlag Basel
Erschienen in
Archivum Immunologiae et Therapiae Experimentalis / Ausgabe 3/2011
Print ISSN: 0004-069X
Elektronische ISSN: 1661-4917
DOI
https://doi.org/10.1007/s00005-011-0125-2