KEGG   DISEASE: Follicular lymphomaHelp
Entry
H01613                      Disease                                

Name
Follicular lymphoma
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
BRITE hierarchy
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]
Carmustine [DR:D00254]
Doxorubicin hydrochloride [DR:D01275]
Rituximab [DR:D02994] (CD20-positive)
Obinutuzumab [DR:D09321]
Idelalisib [DR:D10560]
Interferon alfa-2b [DR:D02745]
Plerixafor [DR:D08971]
Ibritumomab tiuxetan [DR:D04489]
Comment
ICD-O-3: 9690/3 Follicular lymphoma, NOS
ICD-O-3: 9691/3 Follicular lymphoma, grade 2
ICD-O-3: 9695/3 Follicular lymphoma, grade 1
ICD-O-3: 9698/3 Follicular lymphoma, grade 3
Other DBs
ICD-11: 2A80
ICD-10: C82
MeSH: D008224
Reference
PMID:24687887 (gene, drug)
  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 (gene, ICD-O-3)
  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 (gene, drug)
  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
  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 (gene, drug)
  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 (drug)
  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 (drug)
  Authors
Ghielmini M
  Title
Follicular lymphoma.
  Journal
Ann Oncol 21 Suppl 7:vii151-3 (2010)
DOI:10.1093/annonc/mdq287
Reference
PMID:15684317 (drug)
  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

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