Timár L, Budai J, Szigeti R
Acta Paediatr Acad Sci Hung. 1981;22(4):353-7.
A six-month-old male infant, whose elder brother had died of progressive vaccinia due to combined immunodeficiency, contracted varicella-zoster infection, and died of disseminated varicella ten days after onset of the disease. As with his elder brother, the blood levels of immunoglobulins were normal, and specific varicella antibodies appeared in his serum, although in low concentrations. His T-lymphocytes, although their number was subnormal, could be stimulated with phytohaemagglutinin, and production of migration inhibitory factor could also be demonstrated. The necropsy confirmed thymic dysplasia. In addition to histological changes, severe combined immunodeficiency and foci of malignant lymphoma were present. On this basis the disease was classified, according to the WHO recommendations, as severe combined immunodeficiency with B lymphocytes, complicated by malignant lymphoma.
一名六个月大的男婴，其哥哥因联合免疫缺陷死于进行性牛痘，该男婴感染了水痘 - 带状疱疹病毒，并在发病十天后死于播散性水痘。和他哥哥一样，其免疫球蛋白血液水平正常，血清中出现了特异性水痘抗体，不过浓度较低。他的T淋巴细胞数量虽低于正常水平，但能被植物血凝素刺激，且也能证明有迁移抑制因子产生。尸检证实有胸腺发育不良。除了组织学变化外，还存在严重联合免疫缺陷和恶性淋巴瘤病灶。在此基础上，根据世界卫生组织的建议，该病被分类为伴有B淋巴细胞的严重联合免疫缺陷，并伴有恶性淋巴瘤并发症。
