Follicular lymphoma 3034995 225732583 2008-07-15T03:31:53Z WhatamIdoing 1998764 /* Epidemiology */ new section {{Infobox_Disease | Name = {{PAGENAME}} | Image = | Caption = | DiseasesDB = | ICD10 = {{ICD10|C|82||c|81}} | ICD9 = {{ICD9|202.0}} | ICDO = M9690/3 | OMIM = | MedlinePlus = | eMedicineSubj = med | eMedicineTopic = 1362 | MeshID = D008224 | }} '''Follicular lymphoma''' is the most common of the indolent [[non-Hodgkin's lymphoma]]s. It is defined as a [[lymphoma]] of [[lymph follicle|follicle]] center [[B-cells]] ([[centrocytes]] and [[centroblasts]]), which has at least a partially follicular pattern. It is positive for [[CD10]].<ref>[http://pleiad.umdnj.edu/hemepath/follicular/follicular.html Overview] at [[University of Medicine and Dentistry of New Jersey|UMDNJ]]</ref> ==Morphology== The [[tumor]] is composed of follicles containing a mixture of centrocytes (WHO nomenclature) or cleaved follicle center cells (older American nomenclature), "[[small cells]]", and centroblasts (WHO nomenclature) or large noncleaved follicle center cells (older American nomenclature), "[[large cells]]". These follicles are surrounded by non-malignant cells, mostly T-cells. In the follicles, centrocytes typically predominate; centroblasts are usually in minority. According to the WHO criteria, the disease is morphologically graded into grade 1 (<5 centroblasts per high-power field (hpf)), grade 2 (5-15 centroblasts/hpf) and grade 3 (>15 centroblasts/hpf), and grade 3 is further subdivided into grade 3a (centrocytes still present) and the rare grade 3b (the follicles consist almost entirely of centroblasts). The clinical relevance of this grading system is debated, although grades 1, 2 and 3a can be treated as an indolent disease, while grade 3b is an aggressive disease. Occasional cases may show plasmacytoid differentiation or [[foci]] of [[marginal zone]] or [[monocytoid]] B-cells. ==Causes== A [[Chromosomal translocation|translocation]] between [[chromosome]]14 and 18 results in the overexpression of the [[bcl-2]] gene. This overexpression causes a blockage of [[apoptosis]], or programmed cell death. This translocation has been associated with the development of follicular lymphoma. ==Treatment== There is no consensus regarding the best treatment [[algorithm]], but watch-and-wait policies, (combinations of) alkylators and nucleoside analogues, anthracycline-containing regimens (eg. [[CHOP]]), [[rituximab]], autologous and allogeneic [[hematopoietic stem cell transplantation]] have all been applied. The disease is regarded as incurable (although allogeneic stem cell transplanation may be curative, the [[death|mortality]] from the procedure is too high to be a first line option). The exception is localized disease, which can be cured by local [[irradiation]]. The typical pattern is one of good responses from treatment, followed by relapses some years later. [ ==Prognosis== [Median]] survival is around 10 years, but the range is wide, from less than one year, to more than 20 years. Some patients may never need treatment. ==Epidemiology== Of all [[lymphoproliferative disorders|cancers involving the same class of blood cell]], 22% of cases are follicular lymphomas.<ref name="isbn0-7817-5007-5">{{cite book |author=Turgeon, Mary Louise |title=Clinical hematology: theory and procedures |publisher=Lippincott Williams & Wilkins |location=Hagerstwon, MD |year=2005 |pages=283 |isbn=0-7817-5007-5 |quote=Frequency of lymphoid neoplasms. (Source: Modified from WHO Blue Book on Tumour of Hematopoietic and Lymphoid Tissues. 2001, p. 2001.) }}</ref> ==References== <references/> ==External links== * [http://www.LymphomaSurvival.com Strategies for Long-Term Survival] * [http://www.lymphomation.org/type-follicular.htm Follicular Lymphoma Resource page] - Patients Against Lymphoma * [http://www.lymphomainfo.net/nhl/follicular.html Lymphoma information network] [[Category:Hematology]] [[Category:Types of cancer]] {{Hematological malignancy histology}} [[de: Follikuläres Lymphom]]