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Blood, 1 September 2002, Vol. 100, No. 5, pp. 1570-1574
CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
HLA-DR15 (DR2) is overrepresented in myelodysplastic syndrome and
aplastic anemia and predicts a response to immunosuppression in
myelodysplastic syndrome
Yogen Saunthararajah,
Ryotaro Nakamura,
Jun-Mo Nam,
Jamie Robyn,
Fausto Loberiza,
Jaroslaw P. Maciejewski,
Toni Simonis,
Jeffrey Molldrem,
Neal S. Young, and
A. John Barrett
From the University of Illinois at Chicago; National
Heart, Lung, and Blood Institute, Bethesda, MD; National Cancer
Institute, Rockville, MD; International Bone Marrow Transplant
Registry, Milwaukee, WI; HLA Laboratory, Department of Transfusion
Medicine, National Institutes of Health, Bethesda, MD; and MD Anderson
Cancer Center, Houston, TX.
The extent and importance of autoimmune mechanisms in
myelodysplastic syndrome (MDS) and the role of immunosuppression in the
treatment of this disease are not well defined. We report overrepresentation of HLA-DR2 and its serologic split HLA-DR15 in both
MDS and aplastic anemia (AA). Four clinically and ethnically defined
patient groups were analyzed. The HLA-DR15 antigen frequencies among
North American white MDS patients (n = 72) and AA patients (n = 59), who received immunosuppressive treatment at the National Institutes of Health (NIH), were 36% and 42%, respectively. These antigen frequencies were significantly higher than that of the control
population of 240 North American white NIH blood donors typed for HLA
antigens by the same molecular technique (HLA-DR15, 21.3%,
P = .01 for MDS, P < .001 for AA). Among
North American white patients reported in the International Bone Marrow
Transplant Registry (IBMTR), 30% of 341 MDS patients and 33% of 364 AA patients were positive for HLA-DR2. These antigen frequencies were
higher than those reported for the general North American white
population (HLA-DR2, 25.3%, P = .089 for MDS,
P = .01 for AA). The DR15 and DR2 frequencies were
significantly increased in MDS refractory anemia (RA)
(P = .036 and P = .01, respectively) but
not MDS refractory anemia with excess blasts. In the NIH MDS patients,
HLA-DR15 was significantly associated with a clinically relevant
response to antithymocyte globulin (ATG) or cyclosporine
immunosuppression (multivariate analysis, P = .008). In
MDS with RA, DR15 may be useful as a guide to pathophysiology,
prognosis, and treatment.

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