Bernstein M L, Reece E R, de Chadarévian J P, Koch P A
Cancer. 1986 Aug 15;58(4):969-72. doi: 10.1002/1097-0142(19860815)58:4<969::aid-cncr2820580428>3.0.co;2-s.
Lymphomatoid granulomatosis is an unusual disorder histologically characterized by an angiocentric, angiodestructive mixed cellular infiltrate. The most frequent clinical manifestations are seen in the lungs, the skin, and the central nervous system. Progression to lymphoma may occur, particularly in patients who are anergic. The authors report the case of a boy who had had a splenectomy at 2 years of age for presumed Evan's syndrome, and two episodes of pneumococcal meningitis at 5 and 10 years of age. At 14 years, he had severe respiratory compromise, and a lung biopsy specimen showed lymphomatoid granulomatosis. The liver and bone marrow also were affected. Improvement occurred with multiagent chemotherapy, but he had multiple relapses. A bone marrow transplant was performed using a human leukocyte antigen (HLA) identical mixed lymphocyte culture (MLC) nonreactive brother as the donor. He remains in remission more than 3 years post-transplant. In addition, his abnormal immune function has improved.
淋巴瘤样肉芽肿病是一种不常见的疾病，组织学特征为血管中心性、血管破坏性混合细胞浸润。最常见的临床表现见于肺部、皮肤和中枢神经系统。可能会进展为淋巴瘤，尤其是在无反应性的患者中。作者报告了一例男孩的病例，该男孩2岁时因疑似埃文斯综合征接受了脾切除术，5岁和10岁时发生了两次肺炎球菌性脑膜炎。14岁时，他出现严重的呼吸功能不全，肺活检标本显示为淋巴瘤样肉芽肿病。肝脏和骨髓也受到影响。多药化疗后病情有所改善，但他多次复发。使用一名人类白细胞抗原（HLA）相同、混合淋巴细胞培养（MLC）无反应的兄弟作为供体进行了骨髓移植。移植后3年多他一直处于缓解状态。此外，他异常的免疫功能也有所改善。
