Sordillo P P, Epremian B, Koziner B, Lacher M, Lieberman P
Cancer. 1982 May 15;49(10):2070-6. doi: 10.1002/1097-0142(19820515)49:10<2070::aid-cncr2820491019>3.0.co;2-s.
Six patients with lymphomatoid granulomatosis were studied. Two patients had eye involvement, a rare manifestation of this disease. Both had complete disappearance of disease in this area after radiation therapy. One patient presented with an autoimmune hemolytic anemia, which remitted completely with chemotherapy. The use of corticosteroids or chemotherapy was effective in bringing about a decrease in pulmonary infiltrates in all six patients, although the duration of response was limited. One patient did have a prolonged period of clinical remission, and died of an unrelated cause. Evidence of profound immunologic abnormalities was found in these patients by a variety of tests. The extensive immunologic dysfunction present in patients with lymphomatoid granulomatosis has not previously been described. Most strikingly, patients with lymphomatoid granulomatosis have a severe impairment in T-lymphocyte function, confirmed by their inability to react to common skin test antigens or dinitrochlorobenzene, decreased in vitro lymphocyte responsiveness to mitogens and antigens and changes in T-cell subset populations. This may account for the frequent development of malignant lymphomas in patients with this condition.
对6例淋巴瘤样肉芽肿病患者进行了研究。2例患者有眼部受累，这是该疾病的一种罕见表现。放疗后这两个部位的疾病均完全消失。1例患者出现自身免疫性溶血性贫血，化疗后完全缓解。使用皮质类固醇或化疗对所有6例患者肺部浸润的减少均有效，尽管缓解持续时间有限。1例患者确实有较长时间的临床缓解期，最终死于无关原因。通过各种检测在这些患者中发现了严重免疫异常的证据。淋巴瘤样肉芽肿病患者中存在的广泛免疫功能障碍此前尚未见报道。最显著的是，淋巴瘤样肉芽肿病患者的T淋巴细胞功能严重受损，这通过他们对常见皮肤试验抗原或二硝基氯苯无反应、体外淋巴细胞对有丝分裂原和抗原的反应性降低以及T细胞亚群数量的变化得到证实。这可能解释了这种情况下患者恶性淋巴瘤的频繁发生。
