Clinical, biologic, and pathologic features in 157 patients with angioimmunoblastic T-cell lymphoma treated within the Groupe d'Etude des Lymphomes de l'Adulte (GELA) trials
Clinical, biologic, and pathologic features in 157 patients with angioimmunoblastic T-cell lymphoma treated within the Groupe d'Etude des Lymphomes de l'Adulte (GELA) trials
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DOI：
10.1182/blood-2007-08-105759
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发表时间：
2008-05-01
期刊：
影响因子：
20.3
通讯作者：
Gaulard, Philippe
中科院分区：
文献类型：
--
作者：
Mourad, Nathalie;Mounier, Nicolas;Gaulard, Philippe
为了评估血管免疫母细胞性t细胞淋巴瘤（AITL）的临床生物学和病理特征的预后意义，我们从GELA LNH87-LNH93随机临床试验中检索了157例AITL患者。147名患者接受了环磷酰胺、阿霉素、长春新碱和强的松（CHOP）样方案，其中一半患者的疗程加强。组织学分类为“大细胞丰富”41例，“典型”116例（其中上皮样细胞丰富19例，透明细胞丰富14例，生发中心增生4例）。根据阳性淋巴样细胞的丰度，对62例患者进行CD10和CXCL13表达评分。中位年龄为62岁，81%为晚期，72%为B型症状，65%为贫血，50%为高γ -球蛋白血症，66%为LDH升高。总体7年生存率为30%。在多变量分析中，只有男性（P = 0.004）、纵隔淋巴结病（P = 0.041）和贫血（P = 0.042）对总生存率有不利影响。大细胞的增加和高水平的CD10和CXCL13不影响生存。强化治疗没有提高生存率。总之，AITL是一种形态学异质性的t细胞淋巴瘤，通常表达CXCL13和CD10，携带少量预后因素。即使密集治疗，它也预示着预后不良。然而，AITL并不总是致命的，30%的患者在7年存活。
To evaluate the prognostic significance of clinicobiologic and pathological features in angioimmunoblastic T-cell lymphoma (AITL), 157 AITL patients were retrieved from the GELA LNH87-LNH93 randomized clinical trials. One hundred forty-seven patients received a cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP)-like regimen with intensified courses in half of them. Histologically, 41 cases were classified as "rich in large cells" and 116 as "classic" (including 19 rich in epithelioid cells, 14 rich in clear cells, and 4 with hyperplastic germinal centers). Sixty-two cases were scored for CD10 and CXCL13 expression according to the abundance of positive lymphoid cells. Median age was 62 years, with 81% advanced stage, 72% B symptoms, 65% anemia, 50% hypergamma-globulinemia, and 66% elevated LDH. Overall 7-year survival was 30%. In multivariate analysis, only male sex (P = .004), mediastinal lymphadenopathy (P = .041), and anemia (P = .042) adversely affected overall survival. Increase in large cells and high level of CD10 and CXCL13 did not affect survival. Intensive regimen did not improve survival. In conclusion, AITL is a morphologically heterogeneous T-cell lymphoma commonly expressing CXCL13 and CD10 and carrying few prognostic factors. It portends a poor prognosis even when treated intensively. However, AITL is not always lethal with 30% of patients alive at 7 years.