Leukocyte transfusion-associated granulocyte responses in a patient with X-linked hyper-IgM syndrome
Leukocyte transfusion-associated granulocyte responses in a patient with X-linked hyper-IgM syndrome
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DOI：
10.1023/a:1023286807853
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发表时间：
1998-11-01
影响因子：
9.1
通讯作者：
Cooper, MD
中科院分区：
文献类型：
--
作者：
Atkinson, TP;Smith, CA;Cooper, MD
X连锁高IgM综合征（XHIM）是由CD154（CD40配体，gp39）突变引起的严重先天性免疫缺陷，CD154是B细胞上CD40的T细胞配体。慢性或周期性中性粒细胞减少症是一种常见的并发症，可增加对严重感染的易感性。我们描述了一个病人的变种XHIM谁产生血清伊加和IgM水平升高，并患有慢性严重的中性粒细胞减少症。由于粘膜感染，10例白细胞输注中有8例输注了来自姨妈的细胞，导致了类似的内源性粒细胞产生。用突变的CD154蛋白进行的转染研究表明，该蛋白在细胞表面表达，并形成不与CD40相互作用的异常三聚体。这些数据表明，来自患者姑姑的同种异体细胞，可能是携带功能性CD 154的活化T细胞，可能与CD 40（+）受体细胞相互作用，使骨髓中的髓系前体细胞成熟。
X-linked hyper-IgM syndrome (XHIM) is a severe congenital immunodeficiency caused by mutations in CD154 (CD40 ligand, gp39), the T cell ligand for CD40 on B cells. Chronic or cyclic neutropenia is a frequent complicating feature that heightens susceptibility to severe infections. We describe a patient with a variant of XHIM who produced elevated levels of serum IgA as well as IgM and suffered from chronic severe neutropenia. Eight of ten leukocyte transfusions with cells from a maternal aunt, performed because of mucosal infections, resulted in similar episodes of endogenous granulocyte production. Transfection studies with the mutant CD154 protein indicate that the protein is expressed at the cell surface and forms an aberrant trimer that does not interact with CD40. The data suggest that allogeneic cells from the patient's aunt, probably activated T cells bearing functional CD154, may interact with CD40(+) recipient cells to produce maturation of myeloid precursors in the bone marrow.