The frequency of HLA class I alleles in Japanese patients with bone marrow failure
The frequency of HLA class I alleles in Japanese patients with bone marrow failure
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日本骨髓衰竭患者 HLA I 类等位基因的频率
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发表时间：
2006
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通讯作者：
P. Aa
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作者：
P. Aa
再生障碍性贫血患者HLA-B*4002和HLA-A *0206等位基因频率的研究（n=32; 21.9%）和阵发性血小板减少性血红蛋白尿（PNH）（n=24; 22.9%）分别与对照组有显著差异（n=371;分别为8.6%，p<0.002和7.7%，p<0.001），表明AA或PNH中的每个特定等位基因可能与这些疾病的免疫病理生理学有关。自身反应性T淋巴细胞参与骨髓衰竭（BMF）综合征的免疫机制，包括再生障碍性贫血（AA）、阵发性睡眠性血红蛋白尿症（PNH）和骨髓增生异常综合征（MDS）。1为了阐明人类白细胞抗原（HLA）I类等位基因在BMF综合征中的频率和临床意义，我们使用高分辨率基因分型方法对78例日本BMF患者（包括32例AA，24例PNH和22例MDS）进行了这些等位基因的研究。AA的诊断和严重程度分级基于国际粒细胞缺乏症和再生障碍性贫血研究组4和Frickhofen等人的标准，5分别。PNH和MDS的诊断依据国际PNH兴趣小组标准
The frequencies of the HLA-B*4002 and HLAA*0206 alleles in patients with aplastic anemia (AA) (n=32; 21.9%) and paroxysmal nocturanl hemoglobinuria (PNH) (n=24; 22.9%), respectively, were significantly different from those in controls (n=371; 8.6%, p<0.002 and 7.7%, p<0.001, respectively), suggesting that each specific allele in AA or PNH may be related to the immunologic pathophysiology of these disorders. Autoreactive T lymphocytes are implicated in the immune mechanisms involved in the bone marrow failure (BMF) syndrome, including aplastic anemia (AA), paroxysmal nocturnal hemoglobinuria (PNH), and myelodysplastic syndrome (MDS). 1 In order to clarify the frequency and some clinical significance of the human leukocyte antigen (HLA) class I alleles in the BMF syndrome, we investigated these alleles using a high-resolution method of genotyping 2,3 in 78 Japanese patients with BMF, including 32 with AA, 24 with PNH, and 22 with MDS. The diagnosis and grading of the severity of AA were based on the criteria of the International Agranulocytosis and Aplastic Anemia Study Group 4 and that of Frickhofen et al., 5 respectively. The diagnosis of PNH and MDS was made according to the International PNH Interest Group criteria