Blattner W A, Naiman J L, Mann D L, Wimer R S, Dean J H, Fraumeni J F
Ann Intern Med. 1978 Aug;89(2):173-6. doi: 10.7326/0003-4819-89-2-173.
Acute lymphocytic leukemia developed almost simultaneously in two adolescent brothers, and another brother and both parents had rheumatoid arthritis. Laboratory studies uncovered no evidence for an underlying immunodeficiency state in the family. Immunogenetic evaluation showed the leukemic siblings to be HLA- and mixed-leukocyte-culture identical and homozygous for a recessively inherited locus dictating the presence of antigens on the surface of B-cells. This Ia antigen, as detected by sera from mothers of leukemic children, appeared to be mapped within the major histocompatibility region and may be a human analogue to murine immune-response antigens associated with susceptibility to leukemia.
两名青少年兄弟几乎同时患上急性淋巴细胞白血病,另一名兄弟以及父母均患有类风湿性关节炎。实验室研究未发现该家族存在潜在免疫缺陷状态的证据。免疫遗传学评估显示,患白血病的兄弟俩HLA相同,混合淋巴细胞培养结果也相同,且对于一个决定B细胞表面抗原存在的隐性遗传位点呈纯合状态。通过白血病患儿母亲的血清检测到的这种Ia抗原,似乎定位在主要组织相容性区域内,可能是与白血病易感性相关的小鼠免疫反应抗原的人类类似物。