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血管免疫母细胞性淋巴结病

Angio-immunoblastic lymphadenopathy.

作者信息

Jacobs P, Kahn L B, King H S

出版信息

S Afr Med J. 1982 Aug 7;62(7):200-2.

PMID:7101070
Abstract

Two patients presented with a short history of constitutional symptoms including fatigue, weight loss, night sweats and pruritus. Both had hepatosplenomegaly and tender lymphadenopathy, and in each case lymph node biopsy revealed prominent vascularization of the interfollicular zones and the presence of an amorphous eosinophilic fibrillar material, together with many epithelioid histiocytes, immunoblasts and plasma cells. In other areas of the same lymph nodes unequivocal lymphocytic lymphoma was present. The first patient was treated with levamisole, to which there was dramatic response. She subsequently died of septicaemia, and at autopsy was shown to be free of lymphoma. The second patient responded completely to the epipodophyllotoxin VP16-213 and is back at work as a heavy labourer without any residual disease. These 2 cases illustrate that complete clinical remission can be obtained in patients with this disease even after lymphomatous transformation has occurred. Levamisole has the additional attraction of being less immunosuppressive in patients whose immune response is already impaired.

摘要

两名患者出现包括疲劳、体重减轻、盗汗和瘙痒在内的全身性症状,病史较短。两人均有肝脾肿大和压痛性淋巴结病,且在每例中,淋巴结活检均显示滤泡间区血管显著增生,存在无定形嗜酸性纤维物质,同时伴有许多上皮样组织细胞、免疫母细胞和浆细胞。在同一淋巴结的其他区域存在明确的淋巴细胞性淋巴瘤。第一名患者接受左旋咪唑治疗,反应显著。她随后死于败血症,尸检显示无淋巴瘤。第二名患者对表鬼臼毒素VP16 - 213完全缓解,现作为重体力劳动者重返工作岗位,无任何残留疾病。这2例表明,即使发生淋巴瘤转化,这种疾病的患者也可获得完全临床缓解。左旋咪唑还有一个额外的优点,即对免疫反应已受损的患者免疫抑制作用较小。

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