Increased frequency of HLA-DR2 in patients with paroxysmal nocturnal hemoglobinuria and the PNH/aplastic anemia syndrome

DOI PDF 被引用文献19件
  • Jaroslaw P. Maciejewski
    From the Hematology Branch and Office of Biostatistics Research, National Heart, Lung and Blood Institute; and the HLA-Laboratory, Department of Transfusion Medicine, and Department of Laboratory Medicine, Hematology Section, Clinical Center, National Institutes of Health, Bethesda, MD.
  • Dean Follmann
    From the Hematology Branch and Office of Biostatistics Research, National Heart, Lung and Blood Institute; and the HLA-Laboratory, Department of Transfusion Medicine, and Department of Laboratory Medicine, Hematology Section, Clinical Center, National Institutes of Health, Bethesda, MD.
  • Ryotaro Nakamura
    From the Hematology Branch and Office of Biostatistics Research, National Heart, Lung and Blood Institute; and the HLA-Laboratory, Department of Transfusion Medicine, and Department of Laboratory Medicine, Hematology Section, Clinical Center, National Institutes of Health, Bethesda, MD.
  • Yogen Saunthararajah
    From the Hematology Branch and Office of Biostatistics Research, National Heart, Lung and Blood Institute; and the HLA-Laboratory, Department of Transfusion Medicine, and Department of Laboratory Medicine, Hematology Section, Clinical Center, National Institutes of Health, Bethesda, MD.
  • Candido E. Rivera
    From the Hematology Branch and Office of Biostatistics Research, National Heart, Lung and Blood Institute; and the HLA-Laboratory, Department of Transfusion Medicine, and Department of Laboratory Medicine, Hematology Section, Clinical Center, National Institutes of Health, Bethesda, MD.
  • Toni Simonis
    From the Hematology Branch and Office of Biostatistics Research, National Heart, Lung and Blood Institute; and the HLA-Laboratory, Department of Transfusion Medicine, and Department of Laboratory Medicine, Hematology Section, Clinical Center, National Institutes of Health, Bethesda, MD.
  • Kevin E. Brown
    From the Hematology Branch and Office of Biostatistics Research, National Heart, Lung and Blood Institute; and the HLA-Laboratory, Department of Transfusion Medicine, and Department of Laboratory Medicine, Hematology Section, Clinical Center, National Institutes of Health, Bethesda, MD.
  • John A. Barrett
    From the Hematology Branch and Office of Biostatistics Research, National Heart, Lung and Blood Institute; and the HLA-Laboratory, Department of Transfusion Medicine, and Department of Laboratory Medicine, Hematology Section, Clinical Center, National Institutes of Health, Bethesda, MD.
  • Neal S. Young
    From the Hematology Branch and Office of Biostatistics Research, National Heart, Lung and Blood Institute; and the HLA-Laboratory, Department of Transfusion Medicine, and Department of Laboratory Medicine, Hematology Section, Clinical Center, National Institutes of Health, Bethesda, MD.

抄録

<jats:title>Abstract</jats:title> <jats:p>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 HLA-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% of responders 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 HLA and likely related to an immune mechanism.</jats:p>

収録刊行物

  • Blood

    Blood 98 (13), 3513-3519, 2001-12-15

    American Society of Hematology

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