Chevailler A, Ifrah N, Monteiro R C, Keyeux G, Renier G, Lefranc M P, Lesavre P, Hurez D
Laboratoire d'Immunopathologie, CHU, Angers, France.
Nouv Rev Fr Hematol (1978). 1990;32(2):159-64.
We report on an unusual association between partial IgA deficiency and acute lymphoblastic leukemia in a young man. We also report results of the family study of immunoglobulin levels, sIgA B cells, in vitro IgA synthesis and molecular analysis of the structural C alpha genes. The IgA deficiency was present at diagnosis of leukemia prior to any therapy. The indirect arguments for a preexisting IgA deficiency are the absence of improvement of the IgA level during complete remission and especially the finding of a similar partial IgA deficiency in a sister who shared the same HLA haplotype, had a low percentage of sIgA B cells and decreased in vitro IgA production. The mother, who had a normal IgA serum level also had decreased in vitro IgA synthesis. No major structural C alpha gene defect was found. The relationship between acute lymphoblastic leukemia and IgA deficiency remains to be elucidated.
我们报告了一名年轻男性中部分IgA缺乏症与急性淋巴细胞白血病之间的一种不寻常关联。我们还报告了对免疫球蛋白水平、分泌型IgA B细胞、体外IgA合成以及结构Cα基因的分子分析的家族研究结果。IgA缺乏症在白血病诊断时就已存在，且在任何治疗之前。支持预先存在IgA缺乏症的间接证据包括：在完全缓解期间IgA水平未改善，特别是在一位共享相同HLA单倍型、分泌型IgA B细胞百分比低且体外IgA产生减少的姐妹中发现了类似的部分IgA缺乏症。母亲的血清IgA水平正常，但体外IgA合成也减少。未发现主要的结构Cα基因缺陷。急性淋巴细胞白血病与IgA缺乏症之间的关系仍有待阐明。
