Primary and secondary cutaneous CD30+ lymphoproliferative disorders:: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment
Primary and secondary cutaneous CD30+ lymphoproliferative disorders:: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment
复制标题
DOI：
10.1182/blood.v95.12.3653.012k23_3653_3661
复制
发表时间：
2000-06-15
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;Geelen, FAMJ;Willemze, R
为了评价我们的诊断和治疗指南，对219例原发性或继发性皮肤CD 30（+）淋巴增殖性疾病患者的临床和长期随访数据进行了评价。研究组包括118例淋巴瘤样丘疹病患者（LyP;第1组），79例原发性皮肤CD 30（+）大T细胞淋巴瘤患者（LTCL;第2组11例，皮肤及区域淋巴结转移组11例，继发性皮肤CD 30（+）LT CL组11例，LyP患者通常不接受任何特异性治疗，而大多数原发性皮肤CD 30（+）LTCL患者接受放疗或切除治疗。第1组和第2组中接受多药化疗的所有皮肤局限性疾病患者均发生1次或多次皮肤复发。诊断后10年内全身性疾病的计算风险为4%，第1组为16%，第3组为20%（初始治疗后）。疾病相关的5年生存率分别为100%（第1组）、96%（第2组）、91%（第3组）和24%（第4组）。结果证实了这些原发性皮肤CD 30（+）淋巴增生性疾病的良好预后，并强调LyP和原发性皮肤CD 30（+）淋巴瘤是密切相关的条件。他们还指出，皮肤和引流淋巴结部位的CD 30（+）LTCL与原发性皮肤CD 30（+）LTCL（无并发淋巴结受累）具有相似的良好预后。多药化疗仅适用于完全成熟或发展中的皮外疾病患者;从未或很少适用于皮肤局限性CD 30（+）淋巴瘤患者。(C)2000年，美国血液学会。
To evaluate our diagnostic and therapeutic guidelines, clinical and long-term follow-up data of 219 patients with primary or secondary cutaneous CD30(+) lympho proliferative disorders were evaluated. The study group included 118 patients with lymphomatoid papulosis (LyP; group 1), 79 patients with primary cutaneous CD30(+) large T-cell lymphoma (LTCL; group 2), 11 patients with CD30(+) LTCL and skin and regional lymph node involve ment (group 3), and 11 patients with secondary cutaneous CD30(+) LTCL (group 4), Patients with LyP often did not receive any specific treatment, whereas most patients with primary cutaneous CD30(+) LTCL were treated with radiotherapy or excision, All patients with skin-limited disease from groups 1 and 2 who were treated with multiagent chemotherapy had 1 or more skin relapses. The calculated risk for systemic disease within 10 years of diagnosis was 4% for group 1, 16% for group 2, and 20% for group 3 (after initial therapy). Disease-related 5-year-survival rates were 100% (group 1), 96% (group 2), 91% (group 3), and 24% (group 4), respectively. The results confirm the favorable prognoses of these primary cutaneous CD30(+) lymphoproliferative disorders and underscore that LyP and primary cutaneous CD30(+) lymphomas are closely related conditions. They also indicate that CD30(+) LTCL on the skin and in 1 draining lymph node station has a good prognosis similar to that for primary cutaneous CD30(+) LTCL without concurrent lymph node involvement. Multiagent chemotherapy is only indicated for patients with full-blown or developing extracutaneous disease; it is never or rarely indicated for patients with skin-limited CD30(+) lymphomas. (C) 2000 by The American Society of Hematology.