DISEASE: Follicular lymphoma


Entry
H01613                      Disease                                
Name
Follicular lymphoma
  Supergrp
Non-Hodgkin lymphoma [DS:H02418]
Description
Follicular lymphoma (FL) is generally an indolent B cell lymphoproliferative disorder of transformed follicular center B cells. FL is characterized by diffuse lymphoadenopathy, bone marrow involvement, splenomegaly, and less commonly other extranodal sites of involvement. The genetic hallmark of FL, the translocation t(14;18)(q32;q21), results in the constitutive overexpression of the bcl 2 protein, which impairs the normal germinal centre apoptotic programme. Inactivating mutations of MLL2 are found in >80% of FL and interfere with the ability of MLL2 to activate gene transcription through H3K4 methylation. Mutations of other histone modifiers (CREBBP, EZH2, MEF2B, and EP300) are found in ;33%, 27%, 15%, and 9% of FL, respectively.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of haematopoietic and lymphoid tissues
   H01613  Follicular lymphoma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Mature B-cell neoplasms
    2A80  Follicular lymphoma
     H01613  Follicular lymphoma
Tumor markers [br08442.html]
 H01613
Related
pathway
hsa00310  Lysine degradation
Gene
IgH-BCL2 (translocation) [HSA:596] [KO:K02161]
EZH2 (mutation) [HSA:2146] [KO:K11430]
MLL2 (mutation) [HSA:9757 8085] [KO:K14959 K09187]
CREBBP (mutation) [HSA:1387] [KO:K04498]
MEF2B (mutation) [HSA:4207] [KO:K09261]
EP300 (mutation) [HSA:2033] [KO:K04498]
Drug
Chlorambucil [DR:D00266]
Copanlisib hydrochloride hydrate [DR:D10798]
Obinutuzumab [DR:D09321]
Tazemetostat hydrobromide [DR:D11485] (EZH2 mutation positive)
Interferon alfa-2b [DR:D02745]
Lenalidomide [DR:D04687]
Ibritumomab tiuxetan [DR:D04489]
Umbralisib tosylate [DR:D11323]
Rituximab and hyaluronidase [DR:D11393]
Lisocabtagene maraleucel [DR:D11990]
Other DBs
ICD-11: 2A80
ICD-10: C82
MeSH: D008224
Reference
PMID:24687887
  Authors
Freedman A
  Title
Follicular lymphoma: 2014 update on diagnosis and management.
  Journal
Am J Hematol 89:429-36 (2014)
DOI:10.1002/ajh.23674
Reference
PMID:24942941
  Authors
Takata K, Miyata-Takata T, Sato Y, Yoshino T
  Title
Pathology of follicular lymphoma.
  Journal
J Clin Exp Hematop 54:3-9 (2014)
DOI:10.3960/jslrt.54.3
Reference
PMID:26989204
  Authors
Kahl BS, Yang DT
  Title
Follicular lymphoma: evolving therapeutic strategies.
  Journal
Blood 127:2055-63 (2016)
DOI:10.1182/blood-2015-11-624288
Reference
PMID:24577532
  Authors
Hiddemann W, Cheson BD
  Title
How we manage follicular lymphoma.
  Journal
Leukemia 28:1388-95 (2014)
DOI:10.1038/leu.2014.91
Reference
PMID:23023713
  Authors
Kridel R, Sehn LH, Gascoyne RD
  Title
Pathogenesis of follicular lymphoma.
  Journal
J Clin Invest 122:3424-31 (2012)
DOI:10.1172/JCI63186
Reference
PMID:25804839
  Authors
Herold M, Scholz CW, Rothmann F, Hirt C, Lakner V, Naumann R
  Title
Long-term follow-up of rituximab plus first-line mitoxantrone, chlorambucil, prednisolone and interferon-alpha as maintenance therapy in follicular lymphoma.
  Journal
J Cancer Res Clin Oncol 141:1689-95 (2015)
DOI:10.1007/s00432-015-1963-9
Reference
PMID:20943608
  Authors
Ghielmini M
  Title
Follicular lymphoma.
  Journal
Ann Oncol 21 Suppl 7:vii151-3 (2010)
DOI:10.1093/annonc/mdq287
Reference
PMID:15684317
  Authors
Rohatiner AZ, Gregory WM, Peterson B, Borden E, Solal-Celigny P, Hagenbeek A, Fisher RI, Unterhalt M, Arranz R, Chisesi T, Aviles A, Lister TA
  Title
Meta-analysis to evaluate the role of interferon in follicular lymphoma.
  Journal
J Clin Oncol 23:2215-23 (2005)
DOI:10.1200/JCO.2005.06.146

» Japanese version

  All links  
Ontology (1)   
   KEGG BRITE (1)   
Drug (40)   
   KEGG DRUG (40)   
Gene (13)   
   KEGG ORTHOLOGY (6)   
   KEGG GENES (7)   
Literature (8)   
   PubMed (8)   
All databases (62)   

Download RDF