Ellegaard J, Boesen A M
Scand J Haematol. 1976 Jul;17(1):36-43. doi: 10.1111/j.1600-0609.1976.tb02838.x.
A 71-year-old woman presented with acutely developed symptoms of generalized lymphadenopathy, intermittent maculo-papular skin rash, pruritus, weight loss, hepato-splenomegaly, pleural exsudate and alternating breast swellings. The histopathological picture of biopsies from a lymph node and from the skin was diagnostic for immunoblastic lymphadenopathy, and the serum concentrations of IgG and IgA were increased. Delayed cutaneous hypersensitivity reactions to various antigens were totally extinguished and the number of T-lymphocytes in the peripheral blood was consistently very low. The number of both T- and B-lymphocytes further decreased during cytostatic treatment and the patient contracted numerous infections. During intermittent treatment with Levamisole the infectious episodes ceased, the cellular immune response was reestablished and the pathological hyperimmuneglobulinaemia suppressed. It is suggested that the primary immunological defect in this disease could be a failing cellular immunity, and that the hyperplasia and hyper-reactivity of the B-cell system are a secondary phenomenon.
一名71岁女性出现急性全身性淋巴结肿大、间歇性斑丘疹性皮疹、瘙痒、体重减轻、肝脾肿大、胸腔积液及交替性乳房肿胀等症状。取自淋巴结和皮肤活检的组织病理学图像诊断为免疫母细胞性淋巴结病,且血清IgG和IgA浓度升高。对各种抗原的迟发性皮肤过敏反应完全消失,外周血T淋巴细胞数量持续很低。在细胞抑制治疗期间,T淋巴细胞和B淋巴细胞数量进一步减少,患者发生了许多感染。在用左旋咪唑间歇治疗期间,感染发作停止,细胞免疫反应得以重建,病理性高免疫球蛋白血症得到抑制。提示该病的原发性免疫缺陷可能是细胞免疫功能衰竭,而B细胞系统的增生和高反应性是继发性现象。