滤泡性淋巴瘤:给病理学家的最新资讯

Follicular lymphoma: updates for pathologists.

作者信息

Khanlari Mahsa, Chapman Jennifer R

机构信息

Department of Pathology and Hematopathology, St. Jude Children's Research Hospital, Memphis, TN, USA.

Department of Pathology, Division of Hematopathology, University of Miami, Sylvester Comprehensive Cancer Center, and Jackson Memorial Hospitals, Miami, FL, USA.

出版信息

J Pathol Transl Med. 2022 Jan;56(1):1-15. doi: 10.4132/jptm.2021.09.29. Epub 2021 Dec 27.

Abstract

Follicular lymphoma (FL) is the most common indolent B-cell lymphoma and originates from germinal center B-cells (centrocytes and centroblasts) of the lymphoid follicle. Tumorigenesis is believed to initiate early in precursor B-cells in the bone marrow (BM) that acquire the t(14;18)(q32;q21). These cells later migrate to lymph nodes to continue their maturation through the germinal center reaction, at which time they acquire additional genetic and epigeneticabnormalities that promote lymphomagenesis. FLs are heterogeneous in terms of their clinicopathologic features. Most FLs are indolent and clinically characterized by peripheral lymphadenopathy with involvement of the spleen, BM, and peripheral blood in a substantial subset of patients, sometimes accompanied by constitutional symptoms and laboratory abnormalities. Diagnosis is established by the histopathologic identification of a B-cell proliferation usually distributed in an at least partially follicular pattern, typically, but not always, in a lymph node biopsy. The B-cell proliferation is biologically of germinal center cell origin, thus shows an expression of germinal center-associated antigens as detected by immunophenotyping. Although many cases of FLs are typical and histopathologic features are straightforward, the biologic and histopathologic variability of FL is wide, and an accurate diagnosis of FL over this disease spectrum requires knowledge of morphologic variants that can mimic other lymphomas, and rarely non-hematologic malignancies, clinically unique variants, and pitfalls in the interpretation of ancillary studies. The overall survival for most patients is prolonged, but relapses are frequent. The treatment landscape in FL now includes the application of immunotherapy and targeted therapy in addition to chemotherapy.

摘要

滤泡性淋巴瘤(FL)是最常见的惰性B细胞淋巴瘤,起源于淋巴滤泡的生发中心B细胞(中心细胞和成中心细胞)。肿瘤发生被认为始于骨髓(BM)中获得t(14;18)(q32;q21)的前体B细胞的早期阶段。这些细胞随后迁移至淋巴结,通过生发中心反应继续成熟,此时它们会获得促进淋巴瘤发生的其他遗传和表观遗传异常。FL在临床病理特征方面具有异质性。大多数FL是惰性的,临床特征为外周淋巴结肿大,相当一部分患者的脾脏、BM和外周血受累,有时伴有全身症状和实验室异常。通过组织病理学鉴定通常以至少部分滤泡模式分布的B细胞增殖来确诊,典型情况(但并非总是如此)是在淋巴结活检中确诊。B细胞增殖在生物学上起源于生发中心细胞,因此通过免疫表型分析可检测出生发中心相关抗原的表达。尽管许多FL病例具有典型性且组织病理学特征明确,但FL的生物学和组织病理学变异性很大,要在这个疾病谱上准确诊断FL,需要了解可模仿其他淋巴瘤的形态学变异,以及临床上罕见的非血液系统恶性肿瘤、独特变异和辅助检查解读中的陷阱。大多数患者的总生存期延长,但复发频繁。目前FL的治疗格局除了化疗外,还包括免疫疗法和靶向疗法的应用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/840f/8743801/e528a79c26d2/jptm-2021-09-29f1.jpg

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