Follicular lymphoma in young adults: a clinicopathological and molecular study of 200 patients

被引:9
作者
Duarte, Ivison X. [1 ,2 ]
Domeny-Duarte, Pollyanna [1 ]
Wludarski, Sheila C. L. [1 ]
Natkunam, Yasodha [3 ]
Bacchi, Carlos E. [1 ,2 ]
机构
[1] Lab Bacchi Consultoria Patol, BR-18602010 Botucatu, SP, Brazil
[2] Univ Sao Paulo, Dept Pathol, Sao Paulo, Brazil
[3] Stanford Univ, Dept Pathol, Sch Med, Stanford, CA 94305 USA
关键词
follicular lymphoma; immunohistochemistry; indolent lymphoma; non-Hodgkin lymphoma; survival; young adults; CLINICAL-SIGNIFICANCE; GENE TRANSLOCATION; EXPRESSION; SURVIVAL; BCL2; TRANSFORMATION; PROLIFERATION; REARRANGEMENT; CHILDREN; GRADE;
D O I
10.1038/modpathol.2013.50
中图分类号
R36 [病理学];
学科分类号
100104 ;
摘要
Follicular lymphoma is clinically heterogenous, and therefore necessitates the identification of prognostic markers to stratify risk groups and optimize clinical management. It is relatively rare in patients younger than 40 years, and the clinicopathologic characteristics and biological behavior in this age group are poorly understood. In the current study, samples from a cohort of 200 patients between 19 and 40 years were evaluated retrospectively with respect to clinical, histologic, and genetic features. These were then correlated with clinical outcome. The median age at presentation was 35 years with a slight female prepoderance (56%). Most of the cases are presented with nodal disease (90%). Concomitant follicular lymphoma and diffuse large B-cell lymphoma were observed in 7 (4%) patients. Immunohistologic studies showed the expression of CD10 (91%), BCL6 (97%), BCL2 (95%), MUM1/IRF4 (12%), MDM2 (17%), and CD23 (25%). BCL2 rearrangement was present in 74%, and BCL6 in 20%. The estimated overall survival of patients was 13 years (mean). The presence of anemia, elevated lactose dehydrogenase, bone marrow involvement, and high-risk follicular lymphoma international prognostic index correlated with adverse overall survival. Our findings revealed that follicular lymphoma in young adults demonstrate similarities with that of older adults, including the frequency of presentation at various anatomic sites, grade, and adverse prognostic factors.
引用
收藏
页码:1183 / 1196
页数:14
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