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Clinical manifestations, pathologic features, diagnosis, and prognosis of follicular lymphoma

Arnold S Freedman, MD
Jon C Aster, MD
Section Editor
Andrew Lister, MD, FRCP, FRCPath, FRCR
Deputy Editor
Rebecca F Connor, MD


Follicular lymphoma (FL, previously called follicle center lymphoma) is a heterogeneous clinicopathologic entity that includes tumors derived from germinal center B cells, both centrocytes (small cleaved follicular center cells) and centroblasts (large noncleaved follicular center cells). FL virtually always has a growth pattern that is partially follicular, giving it a nodular appearance both grossly and microscopically. It is the second most common subtype of non-Hodgkin lymphoma (NHL) and is the most common of the clinically indolent NHLs, defined as those lymphomas in which survival of the untreated patient is measured in years. (See "Classification of the hematopoietic neoplasms".)

The epidemiology, clinical presentation, pathologic features, diagnosis, and prognosis of FL will be reviewed here. The pathobiology and treatment of this disorder are discussed separately. (See "Pathobiology of follicular lymphoma" and "Initial treatment of limited stage (I/II) follicular lymphoma" and "Initial treatment of advanced stage (III/IV) follicular lymphoma" and "Treatment of relapsed or refractory follicular lymphoma".)


Follicular lymphoma (FL) is one of the most common forms of non-Hodgkin lymphoma (NHL). FL occurs in all races and all geographic locations, but the exact worldwide incidence of FL is not known, as epidemiologic data are limited in some countries by a lack of resources that are needed for case ascertainment and accurate diagnosis. As a consequence, most epidemiologic data come from retrospective analyses of patients treated at major centers in the United States and Europe.

In the United States as a whole, FL accounts for approximately 35 percent of NHLs and has an estimated incidence of 3.18 cases per 100,000 people [1]. The estimated incidence in Europe is 2.18 cases per 100,000 persons per year [2]. The incidence is stable over time, but varies by ethnicity, with the incidence in Whites being more than twice that in Black and Asian populations [1,3,4]. FL appears to be less common in Central and South America, where it accounts for approximately 20 percent of NHL [5]. There is no strong sex predilection. The incidence increases with age; FL most frequently presents in middle-aged individuals and the elderly, with a median age at diagnosis of 65 years [6,7]. Rarely, FL arises in children or adolescents [8-11]. (See 'Children' below.)

While numerous potential risk factors have been proposed, most have not been validated in independent studies. As such, there is a lack of consensus regarding risk factors for the development of FL. A small number of cases appear to be familial [12,13]. While the risk of developing FL is slightly increased among relatives of persons with FL, the absolute risk remains low [14]. Diseases, infectious agents, and drugs or toxins associated with the development of NHL in general are discussed in more detail separately. (See "Clinical presentation and diagnosis of non-Hodgkin lymphoma", section on 'Past history'.)


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Literature review current through: Sep 2016. | This topic last updated: Sep 21, 2016.
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