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Titolo:
Increased frequency of HLA-DR2 in patients with paroxysmal nocturnal hemoglobinuria and the PNH/aplastic anemia syndrome
Autore:
Maciejewski, JP; Follmann, D; Nakamura, R; Saunthararajah, Y; Rivera, CE; Simonis, T; Brown, KE; Barrett, JA; Young, NS;
Indirizzi:
NIH, Hematol Branch, Clin Ctr, Hematol Sect,Dept Lab Med, Bethesda, MD 20892 USA NIH Bethesda MD USA 20892 matol Sect,Dept Lab Med, Bethesda, MD 20892 USA NHLBI, Off Biostat Res, Bethesda, MD USA NHLBI Bethesda MD USANHLBI, Off Biostat Res, Bethesda, MD USA HLA Lab, Dept Transfus Med, Bethesda, MD USA HLA Lab Bethesda MD USAHLA Lab, Dept Transfus Med, Bethesda, MD USA
Titolo Testata:
BLOOD
fascicolo: 13, volume: 98, anno: 2001,
pagine: 3513 - 3519
SICI:
0006-4971(200112)98:13<3513:IFOHIP>2.0.ZU;2-R
Fonte:
ISI
Lingua:
ENG
Soggetto:
DEPENDENT APLASTIC-ANEMIA; T-CELL REPERTOIRE; CLASS-II; INDUCED AGRANULOCYTOSIS; HLA ASSOCIATIONS; BONE-MARROW; SUSCEPTIBILITY; DISEASE; CYCLOSPORINE; ANTIGEN;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
40
Recensione:
Indirizzi per estratti:
Indirizzo: Maciejewski, JP NIH, Hematol Branch, Clin Ctr, Hematol Sect,Dept Lab Med, Bldg 10,Rm 7C108, Bethesda, MD 20892 USA NIH Bldg 10,Rm 7C108 Bethesda MD USA 20892 a, MD 20892 USA
Citazione:
J.P. Maciejewski et al., "Increased frequency of HLA-DR2 in patients with paroxysmal nocturnal hemoglobinuria and the PNH/aplastic anemia syndrome", BLOOD, 98(13), 2001, pp. 3513-3519

Abstract

Many autoimmune diseases are associated with HLA alleles, and such a relationship also has been reported for aplastic anemia (AA). AA and paroxysmal nocturnal hemoglobinuria (PNH) are related clinically, and glycophosphoinositol (GPI)-anchored protein (AP)-deficient cells can be found in many patients with AA. The hypothesis was considered that expansion of a PNH clone may be a marker of immune-mediated disease and its association with HLA alleles was examined. The study involved patients with a primary diagnosis of AA, patients with myelodysplastic syndrome (MDS), and patients with primary PNH. Tests of proportions were used to compare allelic frequencies. For patients with a PNH clone (defined by the presence of GPI-AP-deficient granulocytes), regardless of clinical manifestations, there was a higher than normal incidence of HLA-DR2 (58% versus 28%; z = 4.05). The increased presence of HILA-DR2 was found in all frankly hemolytic PNH and in PNH associated with bone marrow failure (AA/PNH and MDS/PNH). HLA-DR2 was more frequent in AA/PNH (56%) than in AA without a PNH clone (37%; z = 3.36). Analysis of a second cohort of patients with bone marrow failure treated with immunosuppression showed that HLA-DR2 was associated with a hematologic response (50% ofresponders versus 34% of nonresponders; z = 2.69). Both the presence of HLA-DR2 and the PNH clone were independent predictors of response but the size of PNH clone did not correlate with improvement in blood count. The results suggest that clonal expansion of GPI-AP-deficient cells is linked to HLAand likely related to an immune mechanism. (C) 2001 by The American Society of Hematology.

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Documento generato il 22/01/20 alle ore 12:27:16