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外源性凋亡途径:皮肤间变性大CD30+ALK-T细胞淋巴瘤更充分治疗的新潜在“靶点”。

Extrinsic apoptotic pathways: a new potential "target" for more sufficient therapy in a case of cutaneous anaplastic large cd30+ alk-T-cell lymphoma.

作者信息

Tchernev Georgi, Cardoso José Carlos, Arseniev Lubomir, Okamoto Hiroyuki

机构信息

Department of Dermatology, Venereology and Dermatologic Surgery, MVZ Kirchheim, Steingaustrasse 13, 73230 Kirchheim unter Teck, Germany.

出版信息

Indian J Dermatol. 2011 Jan;56(1):87-91. doi: 10.4103/0019-5154.77563.

Abstract

The primary cutaneous T-cell lymphomas (CTCL) represent a clonal T-lymphocyte proliferation infiltrating the skin. CD30(+)T-cell lymphomas present clinically as nodules with a diameter between 1 and 15 cm, mostly in elderly patients. The role of the CD30 molecule in patients suffering from T-cell lymphomas is not completely clear yet. The signal transduction pathway which includes CD30 seems to play a key role in tumor progression. In certain forms of T-cellular lymphomas, the interaction between CD30/CD30-ligand is able to provoke apoptosis of the "tumor lymphocytes". The modern conceptions of the pathogenesis of T-cell lymphomas include disorders in the pathways involved in programmed cellular death and disregulation in the expression of certain of its regulatory molecules. We are presenting an unusual case of a female patient with a primary cutaneous form of CD30(+)/ALK(-) anaplastic large T-cell lymphoma. Upon the introduction of systemic PUVA, (psoralen plus ultraviolet light radiation) combined with beam therapy, a complete remission could be noticed. Eight months later, we observed a local recurrence, which was overcome by CHOP chemotherapy (Cyclophosphamide, Hydroxydaunorubicin (Doxorubicin), Vincristin (Oncovin®), Predniso(lo)n). Six months later, new cutaneous lesions had been noticed again. A new therapeutic hope for the patients with anaplastic large CTCL is actually based on the influence of the activity of the different apoptotic pathways. Death ligands, including tumor necrosis factor (TNF)-α, CD95L/FasL, and TRAIL, mediate also some important safeguard mechanisms against tumor growth in patients with CD30(+) cutaneous anaplastic large T-cell lymphomas and critically contribute to lymphocyte homeostasis.

摘要

原发性皮肤T细胞淋巴瘤(CTCL)是一种克隆性T淋巴细胞增殖并浸润皮肤的疾病。CD30(+)T细胞淋巴瘤临床上表现为直径1至15厘米的结节,多见于老年患者。CD30分子在T细胞淋巴瘤患者中的作用尚不完全清楚。包括CD30在内的信号转导通路似乎在肿瘤进展中起关键作用。在某些形式的T细胞淋巴瘤中,CD30/CD30配体之间的相互作用能够引发“肿瘤淋巴细胞”的凋亡。T细胞淋巴瘤发病机制的现代概念包括程序性细胞死亡相关通路的紊乱以及某些调节分子表达的失调。我们报告一例罕见的原发性皮肤型CD30(+)/ALK(-)间变性大T细胞淋巴瘤女性患者。采用全身补骨脂素加紫外线A辐射(PUVA)联合电子束治疗后,患者实现完全缓解。八个月后,出现局部复发,采用CHOP化疗(环磷酰胺、羟基柔红霉素、长春新碱、泼尼松)控制。六个月后,再次出现新的皮肤病变。间变性大细胞CTCL患者的新治疗希望实际上基于不同凋亡通路活性的影响。包括肿瘤坏死因子(TNF)-α、CD95L/FasL和肿瘤坏死因子相关凋亡诱导配体(TRAIL)在内的死亡配体,在CD30(+)皮肤间变性大T细胞淋巴瘤患者中也介导一些重要的抗肿瘤生长保护机制,并对淋巴细胞稳态起关键作用。

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