Follicular lymphoma (FL)
X Y 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 NA
(Note : for Links provided by Atlas : click)
Written | 2001-03 | Antonio Cuneo, Gianluigi Castoldi |
Section of Haematology, University of Ferrara, Arcispedale S. Anna, Corso Giovecca 203, 44100 Ferrara, Italy | ||
Updated | 2005-12 | Antonio Cuneo, Antonella Russo Rossi, Gianluigi Castoldi |
Hematology Section, Dept. Of Biomedical Sciences, University of Ferrara, 44100 Ferrara Italy |
(Note : for Links provided by Atlas : click)
1. Identity
ICD-Topo | C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS |
ICD-Morpho | 9690/3 Follicular lymphoma; Paediatric follicular lymphoma |
Atlas_Id | 2075 |
2. Clinics and Pathology
Phenotype / cell stem origin | Pan-B antigens test positive. The immunophenotypic profile is CD10+, CD5-, sIg+ and the cell of origin is a germinal centre B-cell that has encountered the antigen. |
Epidemiology | This lymphoma accounts for 30-40% of all lymphomas occurring in the adult population in western countries. Its peak incidence is in the fifth and sixth decade. |
Clinics | |
Pathology | The lymphoma is composed of a mixture of centrocytes and centroblasts with a follicular and diffuse pattern. Lymphoma grading by the number of large cells/centroblasts is recommended: three grades are recognized with incresing number of centroblasts. |
Treatment | Depending on age and stage at presentation it may vary from a "watch and wait" policy in initial stages to multiagent chemotherapy in advanced stages. Immunotherapy using chimeric anti-CD20 monoclonal antibody has an important role in combination with chemotherapy. Radioimmunotherapy has an important role in relapsed or refractory patients. |
Evolution | The majority of patients cannot be cured by chemotherapy and eventually relapse. Histologic switch into high grade lymphoma may occur. A positive impact on long term disease free survival and overall survival is likely to derive from the introduction of monoclonal antibodies in association with multiagent chemotherapy. |
Prognosis | Approximately 60% of the patients presenting with limited disease are alive at 10 years. Patients in stages III and IV were reported to have a median survival in the 8-12 years range |
3. Cytogenetics
Cytogenetics Morphological | Seventy-80% of the cases carry the t(14;18)(q32;q21) as the primary chromosome anomaly. Rare variant translocation t(2;18)(p11;q21) and t(18;22)(q21;q11) were described. Approximately 15% of the cases show a 3q27 break, half of which include the t(3;14)(q27;q32) and the variant translocations t(3;22)(q27;q11) and t(2;3)(p11;q27) |
Cytogenetics Molecular | The incidence of 6q21 deletion and 17p13/p53 deletion (see below) by interphase FISH analysis may be around 60% and 20%, respectively |
Additional anomalies | |
4. Result of the chromosomal anomaly
Description | No fusion protein. The t(14;18) brings about the juxtaposition of BCL-2 with the Ig heavy chain joining segment, with consequent marked overexpression of the BCL2 protein product. The majority of breakpoints on 18q22 fall into two regions: the major breakpoint region (60-70% of the cases) and the minor cluster region (20-25% of the cases). In those cases with 3q27/BCL6 involvement the breakpoints on 3q27 are usually located in a different region with respect to high grade diffuse large cell lymphoma. This region is located 245 and 285 kb 5¹ of BCL6 (alternative breakpoint cluster region) |
Oncogenesis | BCL-2 overexpression prevents cell to die by apoptosis. BCL-2 forms heterodimers with BAX and the relative proportion of BCL-2 to BAX determines the functional activity of BCL-2. In vitro, BCL-2 constitutive expression has a definite role in sustaining cell growth, whereas in vivo, BCL-2 transgenes induce a pattern of polyclonal proliferation of mature B-cells. |
5. Bibliography
LAZ3 rearrangements in non-Hodgkin's lymphoma: correlation with histology, immunophenotype, karyotype, and clinical outcome in 217 patients. |
Bastard C, Deweindt C, Kerckaert JP, Lenormand B, Rossi A, Pezzella F, Fruchart C, Duval C, Monconduit M, Tilly H |
Blood. 1994 ; 83 (9) : 2423-2427. |
PMID 8167331 |
Gain of chromosome 7 marks the progression from indolent to aggressive follicle centre lymphoma and is a common finding in patients with diffuse large B-cell lymphoma: a study by FISH. |
Bernell P, Jacobsson B, Liliemark J, Hjalmar V, Arvidsson I, Hast R |
British journal of haematology. 1998 ; 101 (3) : 487-491. |
PMID 9633892 |
BCL6 alternative translocation breakpoint cluster region associated with follicular lymphoma grade 3B. |
Bosga-Bouwer AG, Haralambieva E, Booman M, Boonstra R, van den Berg A, Schuuring E, van den Berg E, Kluin P, Poppema S |
Genes, chromosomes & cancer. 2005 ; 44 (3) : 301-304. |
PMID 16075463 |
Analysis of PTEN mutations and deletions in B-cell non-Hodgkin's lymphomas. |
Butler MP, Wang SI, Chaganti RS, Parsons R, Dalla-Favera R |
Genes, chromosomes & cancer. 1999 ; 24 (4) : 322-327. |
PMID 10092130 |
Rearrangements of chromosome band 1p36 in non-Hodgkin's lymphoma. |
Dave BJ, Hess MM, Pickering DL, Zaleski DH, Pfeifer AL, Weisenburger DD, Armitage JO, Sanger WG |
Clinical cancer research : an official journal of the American Association for Cancer Research. 1999 ; 5 (6) : 1401-1409. |
PMID 10389925 |
Homozygous deletions at chromosome 9p21 involving p16 and p15 are associated with histologic progression in follicle center lymphoma. |
Elenitoba-Johnson KS, Gascoyne RD, Lim MS, Chhanabai M, Jaffe ES, Raffeld M |
Blood. 1998 ; 91 (12) : 4677-4685. |
PMID 9616165 |
Molecular genetics of malignant lymphoma. |
Gaidano G |
In.. |
World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues: report of the Clinical Advisory Committee meeting-Airlie House, Virginia, November 1997. |
Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J, Lister TA, Bloomfield CD |
Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 1999 ; 17 (12) : 3835-3849. |
PMID 10577857 |
Follicular lymphoma. |
Rohatiner A, Lister TA |
In. : page M. |
Prognostic value of chromosomal abnormalities in follicular lymphoma. |
Tilly H, Rossi A, Stamatoullas A, Lenormand B, Bigorgne C, Kunlin A, Monconduit M, Bastard C |
Blood. 1994 ; 84 (4) : 1043-1049. |
PMID 8049424 |
Follicular lymphoma with a burkitt translocation--predictor of an aggressive clinical course: a case report and review of the literature. |
Voorhees PM, Carder KA, Smith SV, Ayscue LH, Rao KW, Dunphy CH |
Archives of pathology & laboratory medicine. 2004 ; 128 (2) : 210-213. |
PMID 14736281 |
Frequent deletions of 6q23-24 in B-cell non-Hodgkin's lymphomas detected by fluorescence in situ hybridization. |
Zhang Y, Weber-Matthiesen K, Siebert R, Matthiesen P, Schlegelberger B |
Genes, chromosomes & cancer. 1997 ; 18 (4) : 310-313. |
PMID 9087572 |
6. Citation
This paper should be referenced as such : |
Cuneo, A ; Russo, Rossi A ; Castoldi, GL |
Follicular lymphoma (FL) |
Atlas Genet Cytogenet Oncol Haematol. 2006;10(3):175-176. |
Free journal version : [ pdf ] [ DOI ] |
On line version : http://atlasgeneticsoncology.usal.es/classic/Anomalies/FollLymphomID2075.html |
History of this paper: |
Cuneo, A ; Castoldi, GL. Follicular lymphoma (FL). Atlas Genet Cytogenet Oncol Haematol. 2001;5(2):125-126. |
http://documents.irevues.inist.fr/bitstream/handle/2042/37734/03-2001-FollLymphomID2075.pdf |
7. External links
REVIEW articles | automatic search in PubMed |
Last year articles | automatic search in PubMed |
All articles | automatic search in PubMed |
© Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Wed Nov 28 16:17:02 CET 2018 |
For comments and suggestions or contributions, please contact us atlasgeneticsoncology@usal.es.