Whittaker S, Foroni L, Luzzatto L, Lampert I, Amlott P, Munro A, Jones R R
Department of Dermatology, Hammersmith Hospital, Royal Postgraduate Medical School, London.
Q J Med. 1988 Aug;68(256):645-55.
Lymphomatoid granulomatosis and lethal midline granuloma are both characterized histologically by atypical pleomorphic angiocentric infiltrates. Whether these conditions are malignant lymphoproliferative disorders remains controversial. Here we report the results of studies carried out in a patient with coeliac disease, who developed recurrent self-healing subcutaneous nodules with the histological changes of lymphomatoid granulomatosis and an invasive nasal tumour with the histological features of lethal midline granuloma. The patient subsequently also developed an erythrophagocytic syndrome. Immunocytochemical labelling of both cutaneous and nasal lesions demonstrated a predominant population of T cells with a CD4-negative CD8-positive phenotype. Analysis of DNA from cutaneous tissue revealed a discrete rearrangement of the beta and gamma T-cell receptor genes. These findings suggest that lymphomatoid granulomatosis is a clonal T-cell lymphoproliferative disorder and its association with lethal midline granuloma indicates that both conditions may have a common histogenesis.
淋巴瘤样肉芽肿病和致死性中线肉芽肿在组织学上均以非典型多形性血管中心浸润为特征。这些病症是否为恶性淋巴增殖性疾病仍存在争议。在此，我们报告了对一名患有乳糜泻患者的研究结果，该患者出现了具有淋巴瘤样肉芽肿组织学改变的复发性自愈性皮下结节以及具有致死性中线肉芽肿组织学特征的侵袭性鼻肿瘤。该患者随后还发展为噬血细胞综合征。对皮肤和鼻部病变进行免疫细胞化学标记显示，主要为具有CD4阴性CD8阳性表型的T细胞群体。对皮肤组织的DNA分析揭示了β和γT细胞受体基因的离散重排。这些发现表明，淋巴瘤样肉芽肿病是一种克隆性T细胞淋巴增殖性疾病，其与致死性中线肉芽肿的关联表明这两种病症可能具有共同的组织发生学。
