Peripheral T-cell lymphomas unspecified presenting in the skin: analysis of prognostic factors in a group of 82 patients
Peripheral T-cell lymphomas unspecified presenting in the skin: analysis of prognostic factors in a group of 82 patients
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DOI：
10.1182/blood-2002-07-1960
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发表时间：
2003-09-15
期刊：
影响因子：
20.3
通讯作者：
Willemze, R
中科院分区：
文献类型：
--
作者：
Bekkenk, MW;Vermeer, MH;Willemze, R
在本研究中，我们对82例皮肤CD30（-）外周t细胞淋巴瘤的临床病理和免疫表型特征进行了评估。本研究的目的是发现基于细胞大小、表型或仅伴有皮肤病变的淋巴瘤的细分是否与临床相关。研究组包括46例原发性皮肤CD30（-）大细胞淋巴瘤和17例小/中型t细胞淋巴瘤以及17例外周血t细胞淋巴瘤，诊断时伴有皮肤和皮外疾病。原发性皮肤小或中型t细胞淋巴瘤患者的预后（5年总生存率为45%）明显优于原发性皮肤CD30（-）大t细胞淋巴瘤患者（12%）和并发皮外疾病患者（12%）。原发性皮肤小或中型t细胞淋巴瘤的预后良好，特别是在表现为CD3(+)CD4(+)CD8（-）表型的局部皮肤病变的患者中发现。在原发性皮肤t细胞淋巴瘤（CTCL）组和并发组中，皮肤病变的程度和表型对生存没有任何影响。我们的研究结果表明，无论诊断时是否存在皮外疾病、细胞大小和CD4（+）或CD8（+）表型的表达，未指明的外周血t细胞淋巴瘤在皮肤中表现为不良预后。唯一的例外是一组原发性皮肤小或中型多形性ctcl，具有CD3(+)CD4(+)CD8（-）表型，并表现为局部皮肤病变。(C) 2003年由美国血液病学会出版。
In the present study the clinicopathologic and immunophenotypic features of 82 patients with a CD30(-) peripheral T-cell lymphoma, unspecified, presenting in the skin were evaluated. The purpose of this study was to find out whether subdivision of these lymphomas on the basis of cell size, phenotype, or presentation with only skin lesions is clinically relevant. The study group included 46 primary cutaneous CD30(-) large cell lymphomas and 17 small/medium-sized T-cell lymphomas as well as 17 peripheral T-cell lymphomas with both skin and extracutaneous disease at the time of diagnosis. Patients with primary cutaneous small- or medium-sized T-cell lymphomas had a significantly better prognosis (5-year-overall survival, 45%) than patients with primary cutaneous CD30(-) large T-cell lymphomas (12%) and patients presenting with concurrent extracutaneous disease (12%). The favorable prognosis in this group with primary cutaneous small- or medium-sized T-cell lymphomas was particularly found in patients presenting with localized skin lesions expressing a CD3(+)CD4(+)CD8(-) phenotype. In the primary cutaneous T-cell lymphoma (CTCL) group and in the concurrent group, neither extent of skin lesions nor phenotype had any effect on survival. Our results indicate that peripheral T-cell lymphomas, unspecified, presenting in the skin have an unfavorable prognosis, irrespective of the presence or absence of extracutaneous disease at the time of diagnosis, cell size, and expression of a CD4(+) or CD8(+) phenotype. The only exception was a group of primary cutaneous small- or medium-sized pleomorphic CTCLs with a CD3(+)CD4(+)CD8(-) phenotype and presenting with localized skin lesions. (C) 2003 by The American Society of Hematology.