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Prepublished online as a Blood First Edition Paper on November 27, 2002; DOI 10.1182/blood-2002-09-2791.
Submitted September 12, 2002
Accepted November 19, 2002
Distinct HLA Associations by Stroke Subtype in Children with Sickle Cell Anemia
Carolyn Hoppe*, William Klitz, Janelle Noble, Lara Vigil, Elliott Vichinsky, and Lori Styles
Hematology/Oncology, Children's Hospital Oakland, Oakland, CA, USA
Children's Hospital Oakland Research Institute, Oakland, CA, USA
* Corresponding author; email: choppe{at}mail.cho.org.
Children with sickle cell anemia (SCA) carry a 200-fold increased risk of cerebral infarction. Stroke can be the result of small vessel (SV) and/or large vessel (LV) disease. However, it is unknown whether these subtypes result from the same pathophysiologic processes. Complete HLA genotyping was performed on 231 eligible children previously enrolled in the CSSCD. Cerebral infarction on MRI was documented in 71 patients and 160 patients had a negative MRI. Based on MRI/MRA findings, infarct size, and location, 36 patients were classified as having LV stroke and 35 as having SV stroke. When comparing the total MRI-positive group with the MRI-negative group, HLA DPB1*0401 was associated with increased stroke risk ( p=.01), while DPB1*1701 ( p=.02) conferred protection from stroke. These DPB1 associations with stroke were attributed to the SV stroke group, where DPB1*0401 was associated with susceptibility (p=.003) and DPB1*1701 with protection from stroke(p=.06). In the LV stroke subgroup, HLA-A*0102 (p=.02) and A*2612 (p=.007) conferred susceptibility, whereas A*3301(p=.04) protected from stroke. These results suggest that specific HLA alleles influence stroke risk and appear to contribute differently to SV and LV stroke subtypes. The distinct HLA associations with SV and LV stroke suggest that different pathologic processes may be involved in the development of stroke in children with SCA. If these results are confirmed in a larger study, HLA type may serve as a useful marker for early identification of SCA patients at high risk for stroke.
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