Clinicopathologic analysis of follicular lymphoma occurring in children
Clinicopathologic analysis of follicular lymphoma occurring in children
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DOI：
10.1182/blood.v99.6.1959
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发表时间：
2002-03-15
期刊：
影响因子：
20.3
通讯作者：
Behm, FG
中科院分区：
文献类型：
--
作者：
Lorsbach, RB;Shay-Seymore, D;Behm, FG
滤泡性淋巴瘤是一种少见的儿童淋巴系统恶性肿瘤，因此在免疫表型和分子发病机制方面仍缺乏特征性。23例儿童滤泡性淋巴瘤患者，平均年龄11岁，男女之比为2.3：1。在19例有临床特征的患者中，15例为I期，1例为II期，3例为III或IV期。所有肿瘤均为滤泡状结构，74%的病例具有2~3级的组织学特征。所有患者均表达CD20和bcl6，其中CD10阳性率为80%。16例中只有5例表达bcl2。与这一发现一致的是，聚合酶链式反应仅在16例中的2例检测到bcl2基因重排。这些患者主要接受环磷酰胺、阿霉素、长春新碱和强的松为主的化疗；4例患者还接受了受累野放疗。在可获得临床随访的13例患者中，除2例外，其余患者均获得持久的临床缓解。重要的是，4例bcl2弥漫性阳性的患者要么表现为III/IV期疾病，要么表现为难治性疾病，而bcl2阴性的肿瘤患者均为I期疾病，完全缓解，无复发。这些发现表明，与成人滤泡性淋巴瘤相比，bcl2表达异常在大多数儿童滤泡性淋巴瘤中并不起重要的致病作用。然而，bcl2在儿童滤泡性淋巴瘤中的表达确定了一组患者的亚群，在这些患者中，疾病经常在临床上扩散，对联合化疗更难治。
Follicular lymphoma is a rare lymphoid malignancy in pediatric patients and consequently remains poorly characterized, particularly with respect to its immunophenotype and molecular pathogenesis. A total of 23 pediatric patients with follicular lymphoma were identified, with a median age of 11 years and a male-to-female ratio of 2.3:1. Of the 19 patients for whom presenting clinical features were available, 15 patients had stage I,1 had stage II, and 3 had stage III or IV disease. All tumors had a follicular architecture, and 74% of cases had grade 2 or 3 histologic features. All patients expressed CD20 and bcl-6, and 80% were positive for CD10. Bcl-2 expression was detected in only 5 of 16 cases. Consistent with this finding, bcl-2 gene rearrangements were detected in only 2 of 16 cases by polymerase chain reaction. These patients were treated primarily with cyclophosphamide, doxorubicin, vincristine, and prednisone-based chemotherapy; 4 patients also received involved-field irradiation. Of the 13 patients with available clinical follow-up, all but 2 achieved durable clinical remission. Importantly, all 4 patients with tumors diffusely positive for bcl-2 either presented with stage III/IV disease or had disease refractory to therapy, whereas patients with bcl-2-negative tumors uniformly had stage I disease, achieved complete remission, and experienced no relapses. These findings indicate that, in contrast to adult follicular lymphomas, dysregulated bcl-2 expression does not play a significant pathogenetic role in most pediatric follicular lymphomas. However, bcl-2 expression in pediatric follicular lymphoma identifies a subset of patients in whom disease is often disseminated at clinical presentation and is more refractory to combination chemotherapy.