Hansen J A, Good R A, Dupont B
Transplantation. 1977 Apr;23(4):366-74. doi: 10.1097/00007890-197704000-00011.
Weak or weak intermediate reactions in one-way mixed lymphocyte culture (MLC) were seen between a patient and at least one parent in the families of 6 of 15 patients with severe combined immunodeficiency disease, 3 of 4 patients with Fanconi's anemia, and 3 of 7 patients with congenital neutropenia (CN). In control family material, weak MLC reactions were seen in 1.4 per cent (4 of 285) of individual parent-child and child-parent combinations or in 2.1 per cent (3 of 143) of the total number of parent-child pairs. The increase in frequency of weak MLC reactions seen in the familes of patients with severe combined immunodeficiency disease and Fanconi's anemia occurred most frequently between mother and patient. This finding could be relevant to the pathogenesis of these diseases. In children with CN, the disease seems to be associated with the HLA antigen B12; in addition, two of the patients with CN appear to be homozygous for HLA-D. Because of the relatively frequent compatibility seen in MLC reactions between parents and children with severe combined immunodeficiency disease, Fanconi's anemia, and CN, it is suggested that those parents could be potential donors for bone marrow transplantation.
在15例重症联合免疫缺陷病患者中的6例、4例范可尼贫血患者中的3例以及7例先天性中性粒细胞减少症（CN）患者中的3例的家族中，患者与至少一位父母之间在单向混合淋巴细胞培养（MLC）中出现了弱或弱中等反应。在对照家族材料中，在1.4%（285例中的4例）的亲子和子亲组合中或在2.1%（143对亲子对中的3对）的亲子对总数中观察到了弱MLC反应。在重症联合免疫缺陷病和范可尼贫血患者家族中观察到的弱MLC反应频率增加最常发生在母亲与患者之间。这一发现可能与这些疾病的发病机制有关。在CN患儿中，该疾病似乎与HLA抗原B12相关；此外，2例CN患者似乎是HLA-D纯合子。由于在重症联合免疫缺陷病、范可尼贫血和CN患者及其父母之间的MLC反应中观察到相对频繁的相容性，因此建议这些父母可能是骨髓移植的潜在供体。
