Blastic variant of mantle cell lymphoma:: a rare but highly aggressive subtype
Blastic variant of mantle cell lymphoma:: a rare but highly aggressive subtype
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DOI：
10.1038/sj.leu.2402272
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发表时间：
2001-11-01
期刊：
影响因子：
11.4
通讯作者：
Lamy, T
中科院分区：
文献类型：
--
作者：
Bernard, M;Gressin, R;Lamy, T
套细胞淋巴瘤(MCL)的原始变异型(BV)被认为是非霍奇金淋巴瘤(NHL)的一种侵袭性很强的亚型。为了确定其临床生物学特征和治疗反应，我们研究了187例MCL患者中33例(17%)的临床生物学特征和治疗反应。根据病理组织学类型、免疫表型、bcl1基因重排和/或细胞周期蛋白DI过表达，诊断为母细胞性变异。3名最初诊断为大细胞非霍奇金淋巴瘤的患者被归类为BV。患者接受一线治疗，包括CHOP-like方案或CVP(n=29)，或氯氨丁苯(n=4)+CHOP或ESAP作为二线治疗。大剂量强化联合干细胞移植(SCT)11例，其中自体SCT 8例，异基因SCT 3例。移植时，除2例患者外，其余患者均完全缓解(CRI，n=5；CR2，n=4)。临床和生物学特征与常见类型的MCL相似。中位年龄为62岁(29-80岁)，性别比(MIF)为2.6：1。在33例患者中，66%有结外部位受累，85%有Ann Arbor分期IV期，82%有周围淋巴结病变。48%的病例可见循环淋巴瘤细胞。12名患者(36%)进入CRI，中位持续时间为11个月。15名患者(46%)无反应，迅速死于进展性疾病。二线治疗的CR2发生率为26%(6/23)。9名患者在大剂量治疗后复发。33例患者中有22例(66%)死于顽固性或进展性疾病。33例BV患者的中位总生存期(OS)为14.5个月，而154MCL患者的中位总生存期为53个月，P<；0.0001。在单因素分析中，OS受年龄、结外部位受累、循环淋巴瘤细胞和国际预后指数(IPI)的影响。在多变量分析中，只有IPI影响OS：IPI大于或等于2的患者的中位OS为8个月，而IPI患者的中位OS为36个月
The blastic variant (BV) form of mantle cell lymphoma (MCL) Is considered to be a very aggressive subtype of non-Hodgkin's lymphoma (NHL). In order to determine Its clinico-biological features and response to therapy we studied 33 patients (17%) out of 187 suffering from MCL who were diagnosed with a BV of MCL. Blastic variant was diagnosed according to histopathological patterns, Immunophenotyping, and bcl1 gene rearrangement and/or cyclin DI overexpression. Three patients Initially diagnosed with large cell NHL were classified as BV. Patients received front-line therapy including CHOP-like regimen or CVP (n = 29), or chlorambucil (n = 4) and CHOP or ESAP as second-line therapy. High-dose Intensification with stem cell transplantation (SCT) was performed in 11 cases (autoSCT, n = 8; alloSCT, n = 3). All but two patients were in complete remission (CR) at the time of transplant (CRI, n = 5; CR2, n = 4). Clinical and biological characteristics did not differ from those of the common form of MCL. The median age was 62 years (29-80), with a sex ratio (MIF) of 2.6:1. Of the 33 patients, 66% had extranodal site Involvement, 85% had an Ann Arbor stage IV, and 82% had peripheral lymphadenopathy. Circulating lymphomatous cells were seen in 48% of cases. Twelve patients (36%) entered a CRI with a median duration of 11 months. Fifteen patients (46%) failed to respond and rapidly died of progressive disease. Second-line therapy led to a 26% (6/23) CR2 rate. Nine patients relapsed after high-dose therapy. Twenty-two of the 33 patients (66%) died of refractory or progressive disease. Median overall survival (OS) time was 14.5 months for the 33 BV patients as compared to 53 months for the 154 patients with a common form of MCL, P < 0.0001. In the univariate analysis, OS was influenced by age, extranodal site involvement, circulating lymphomatous cells, and international prognosis Index (IPI). In the multivariate analysis, only IPI affected OS: patients with IPI greater than or equal to2 had 8 months median OS as compared to 36 months median OS for patients with IPI