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皮肤T细胞淋巴瘤: Sézary综合征、蕈样肉芽肿及相关疾病。

Cutaneous T-cell lymphomas: the Sézary syndrome, mycosis fungoides, and related disorders.

作者信息

Lutzner M, Edelson R, Schein P, Green I, Kirkpatrick C, Ahmed A

出版信息

Ann Intern Med. 1975 Oct;83(4):534-52. doi: 10.7326/0003-4819-83-4-534.

DOI:10.7326/0003-4819-83-4-534
PMID:126656
Abstract

Substantial evidence has accumulated to indicate not only that mycosis fungoides and the Sézary syndrome are closely related malignancies, but to suggest that they are part of a larger spectrum of cutaneous lymphomas. The neoplastic cells of these disorders have membrane features of thymus-derived (T) lymphocytes, a characteristic tissue distribution (skin infiltration, marrow sparing, localization in T-cell regions of lymphoid tissue), and distinctive morphology. For these reasons, we suggest that these lymphoproliferative disorders be grouped together as "cutaneous T-cell lymphomas". The anergy noted in patients of this group with leukemia probably is related to both decreased percentages of normal T cells and presence in the serum of macrophage migration inhibitory activity. Leukapheresis has been particularly effective in the management of selected patients. The homogeneous T-cell populations in the patients with leukemia also provide important opportunities to study many aspects of lymphocyte physiology that are of broad biologic significance.

摘要

大量证据已积累表明,蕈样肉芽肿和塞扎里综合征不仅是密切相关的恶性肿瘤,而且提示它们是更大范围皮肤淋巴瘤的一部分。这些疾病的肿瘤细胞具有胸腺来源(T)淋巴细胞的膜特征、特征性的组织分布(皮肤浸润、骨髓 spared、定位于淋巴组织的T细胞区域)以及独特的形态。基于这些原因,我们建议将这些淋巴增殖性疾病归为“皮肤T细胞淋巴瘤”。该组白血病患者中观察到的无反应性可能与正常T细胞百分比降低以及血清中巨噬细胞移动抑制活性的存在有关。白细胞去除术在选定患者的治疗中特别有效。白血病患者中均匀的T细胞群体也为研究淋巴细胞生理学的许多具有广泛生物学意义的方面提供了重要机会。

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Cutaneous T-cell lymphomas: the Sézary syndrome, mycosis fungoides, and related disorders.皮肤T细胞淋巴瘤: Sézary综合征、蕈样肉芽肿及相关疾病。
Ann Intern Med. 1975 Oct;83(4):534-52. doi: 10.7326/0003-4819-83-4-534.
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[Immunocytological study of cutaneous malignant lymphomas. Classification (author's transl)].皮肤恶性淋巴瘤的免疫细胞学研究。分类(作者译)
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Mayo Clin Proc. 1974 Aug;49(8):575-83.

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