Transformation of mycosis fungoides: clinicopathological and prognostic features of 45 cases. French Study Group of Cutaneious Lymphomas.

Transformation of mycosis fungoides: clinicopathological and prognostic features of 45 cases. French Study Group of Cutaneious Lymphomas.
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蕈样肉芽肿转化:45例临床病理及预后特征。

DOI:
10.1182/blood.v95.7.2212
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发表时间:
2000
期刊:
影响因子:
20.3
通讯作者:
J. Wechsler
J. Wechsler
中科院分区:
医学1区
文献类型:
--
作者:
B. Vergier;A. Muret;M. Beylot‐Barry;L. Vaillant;D. Ekouevi;G. Chêne;A. Carlotti;N. Franck;P. Dechelotte;P. Souteyrand;P. Courville;Pascal Joly;M. Delaunay;M. Bagot;F. Grange;S. Fraitag;J. Bosq;T. Petrella;A. Durlach;A. Mascarel;J. Merlio;J. Wechsler

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蕈样肉芽肿 (MF) 的病程是惰性的,除非发生转化为大 T 细胞淋巴瘤。转化性 MF (T-MF) 的诊断依赖于 MF 病变活检中存在超过 25% 的大细胞。我们分析了法国皮肤淋巴瘤研究组记录的 45 名 T-MF 患者,以更好地确定 MF 转化的临床病理特征并分析其对预后的影响。从 MF 诊断到转化的中位时间为 6.5 年。 20 名患者出现皮外进展。从转化到死亡的平均生存期为 22 个月。在单变量分析中,只有皮外进展与较差的预后相关(5 年精算生存率:7.8% 与 32%)。性别、年龄、转化时的临床和皮肤病阶段、转化速度、大细胞百分比或 CD30 表达(45 个中的 14 个)都没有预后价值。在进行多变量分析时,发现年龄(至少 60 岁)和皮外扩散与不良预后相关。 T-MF 患者和多形性大 T 细胞 CD30- 淋巴瘤患者的生存曲线没有差异。主要的诊断缺陷是“富含组织细胞”的 MF,需要 CD68 染色来诊断 T-MF。 45 名患者中,6 名在临床进展前出现组织学转变,表明可以通过组织学随访进行早期组织病理学诊断。这种早期组织病理学诊断的预后价值必须通过前瞻性研究来证实。
The course of mycosis fungoides (MF) is indolent except when transformation to a large T-cell lymphoma occurs. The diagnosis of transformed MF (T-MF) relies on the presence of more than 25% of large cells on biopsy of an MF lesion. We analyzed 45 patients with T-MF recorded by the French Study Group on Cutaneous Lymphomas to better determine clinicopathological features of MF transformation and to analyze their impact on prognosis. Median time from diagnosis of MF to transformation was 6.5 years. Extracutaneous progression was present in 20 patients. Mean survival from transformation to death was 22 months. In univariate analysis, only an extracutaneous progression was associated with a worse prognosis (5-year actuarial survival: 7.8% versus 32%). Neither sex, age, clinical and skin disease stage at transformation, transformation speed, nor percentage of large cells or CD30 expression (14 of 45) had a prognostic value. When performing multivariate analysis, age (at least 60 years), and extracutaneous spreading were found to be associated with a poor prognosis. There was no difference between survival curves of patients with T-MF and with pleomorphic large T-cell CD30- lymphomas. The main diagnostic pitfall was "histiocytic-rich" MF, requiring CD68 staining for the diagnosis of T-MF. Out of 45 patients, 6 presented an histologic transformation before clinical progression, suggesting that an early histopathological diagnosis may be performed by histological follow-up. The prognostic value of such early histopathological diagnosis must be confirmed by prospective studies.
DOI: --
发表时间: 1988
期刊: The American journal of pathology
影响因子: --
作者:
Salhany,KE;Cousar,JB;Greer,JP;Casey,TT;Fields,JP;Collins,RD
通讯作者: Collins,RD
DOI: 10.1046/j.1523-1747.1998.00167.x
发表时间: 1998-05-01
影响因子: 6.5
作者:
Li, GQ;Chooback, L;Salhany, KE
通讯作者: Salhany, KE
蕈样肉芽肿的转化:T细胞受体β基因分析表明斑块型蕈样肉芽肿和CD30大细胞淋巴瘤有共同的克隆起源。
DOI: 10.1111/1523-1747.ep12365416
发表时间: 1993
期刊: The Journal of investigative dermatology
影响因子: --
作者:
Wood,GS;Bahler,DW;Hoppe,RT;Warnke,RA;Sklar,JL;Levy,R
通讯作者: Levy,R