Follicular lymphoma: 2015 update on diagnosis and management
Follicular lymphoma: 2015 update on diagnosis and management
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DOI：
10.1002/ajh.24200
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发表时间：
2015-12-01
影响因子：
12.8
通讯作者：
Freedman, Arnold
中科院分区：
文献类型：
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作者：
Freedman, Arnold
疾病概述：滤泡性淋巴瘤通常是一种由滤泡中心B细胞转化而成的惰性B细胞增生性疾病。滤泡性淋巴瘤（FL）以弥漫性淋巴腺病、骨髓受累、脾肿大为特征，结外受累部位较少见。一般情况下，细胞减少可发生，但发热、盗汗和体重减轻等体质症状并不常见。诊断：诊断基于组织学，最好是淋巴结活检。在几乎所有病例中，细胞表面CD19、CD20、CD10和单克隆免疫球蛋白以及细胞质中bcl-2蛋白的表达均呈阳性。绝大多数病例具有涉及IgH/bcl-2基因的特征性t（14;18）易位。风险分层：滤泡性淋巴瘤国际预后指数（Follicular Lymphoma International Prognostic Index）预测滤泡性淋巴瘤的预后模型使用了5个独立的预后指标：年龄bbbb60岁、血红蛋白正常、Ann Arbor期III/IV期、累及淋巴结数量bbbb4。0、1、2和>= 3个不良因素的存在定义了低、中、高风险疾病。随着更现代疗法的使用，结果有所改善。风险适应治疗：对于无症状的低体积疾病且无细胞减少的患者，无论是化疗还是单独使用利妥昔单抗，早期治疗都没有生存优势，观察仍然足够。对于需要治疗的患者，大多数患者采用化疗加利妥昔单抗治疗，提高了反应率、反应持续时间和总生存期。随机研究表明，在化疗后维持利妥昔单抗和单药利妥昔单抗均有额外的益处。实验疗法以及干细胞移植（SCT）被认为是复发性疾病。(C) 2015 Wiley期刊公司
Disease overview: Follicular lymphoma is generally an indolent B cell lymphoproliferative disorder of transformed follicular center B cells. Follicular lymphoma (FL) is characterized by diffuse lymphoadenopathy, bone marrow involvement, splenomegaly, and less commonly other extranodal sites of involvement. In general, cytopenias can occur but constitutional symptoms of fever, night sweats, and weight loss are uncommon.Diagnosis: Diagnosis is based on histology of preferably a biopsy of a lymph node. Immunohistochemical staining is positive in virtually all cases for cell surface CD19, CD20, CD10, and monoclonal immunoglobulin, as well as cytoplasmic expression of bcl-2 protein. The overwhelming majority of cases have the characteristic t(14;18) translocation involving the IgH/bcl-2 genes.Risk stratification: The Follicular Lymphoma International Prognostic Index prognostic model for FL uses five independent predictors of inferior survival: age >60 years, hemoglobin normal, Ann Arbor stage III/IV, number of involved nodal areas >4. The presence of 0, 1, 2, and >= 3 adverse factors defines low, intermediate, and high-risk disease. With the use of more modern therapies, outcomes have improved.Risk-adapted therapy: Observation continues to be adequate for asymptomatic patients with low bulk disease and no cytopenias, with no survival advantage for early treatment with either chemotherapy or rituximab alone. For patients needing therapy, most patients are treated with chemotherapy plus rituximab, which has improved response rates, duration of response and overall survival. Randomized studies have shown additional benefit for maintenance rituximab both following chemotherapy-rituximab and single agent rituximab. Experimental therapies as well as stem cell transplantation (SCT) are considered for recurrent disease. (C) 2015 Wiley Periodicals, Inc.