KEGG   DISEASE: Mantle cell lymphoma
Entry
H01464                      Disease                                

Name
Mantle cell lymphoma
  Supergrp
Non-Hodgkin lymphoma [DS:H02418]
Description
Mantle cell lymphoma (MCL) is a B-cell non-Hodgkin lymphoma (NHL) and it accounts for about 6% of all NHL cases. Diagnosis is based on lymph node, bone marrow, or tissue morphology of centrocytic lymphocytes, small cell type, or blastoid variant cells. The molecular hallmark and putative initiating oncogenic event in MCL is the t(11;14)(q13;q32) translocation resulting in constitutional overexpression of cyclin D1 and cell cycle dysregulation in almost all cases. Clinically, MCL shows an aggressive clinical course with a continuous relapse pattern and a median survival of only 3-5 years. However, recently a subset of 15% long-term survivors has been identified with a rather indolent clinical course.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of haematopoietic and lymphoid tissues
   H01464  Mantle cell lymphoma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Mature B-cell neoplasms
    2A85  Other specified mature B-cell neoplasms or lymphoma
     H01464  Mantle cell lymphoma
Tumor markers [br08442.html]
 H01464
Gene
Cyclin D1 (overexpression) [HSA:595] [KO:K04503]
p16/INK4a (deletion) [HSA:1029] [KO:K06621]
p53 (mutation) [HSA:7157] [KO:K04451]
Drug
Ibrutinib [DR:D10223]
Acalabrutinib [DR:D10893]
Bortezomib [DR:D03150]
Lenalidomide [DR:D04687]
Zanubrutinib [DR:D11422]
Brexucabtagene autoleucel [DR:D11880]
Comment
ICD-O-3.1: 9673/3 Mantle cell lymphoma
Other DBs
ICD-11: 2A85.5
ICD-10: C83
MeSH: D020522
Reference
PMID:26103436 (description, gene, drug)
  Authors
Vose JM
  Title
Mantle cell lymphoma: 2015 update on diagnosis, risk-stratification, and clinical management.
  Journal
Am J Hematol 90:739-45 (2015)
DOI:10.1002/ajh.24094
Reference
  Authors
Wang Y, Ma S
  Title
Risk factors for etiology and prognosis of mantle cell lymphoma.
  Journal
Expert Rev Hematol 7:233-43 (2014)
DOI:10.1586/17474086.2014.889561
Reference
PMID:26755518 (description, gene, drug)
  Authors
Cheah CY, Seymour JF, Wang ML
  Title
Mantle Cell Lymphoma.
  Journal
J Clin Oncol 34:1256-69 (2016)
DOI:10.1200/JCO.2015.63.5904
Reference
PMID:24857076 (description, gene, drug)
  Authors
Dreyling M
  Title
Mantle cell lymphoma: biology, clinical presentation, and therapeutic approaches.
  Journal
Am Soc Clin Oncol Educ Book 191-8 (2014)
DOI:10.14694/EdBook_AM.2014.34.191
Reference
PMID:24974852 (drug)
  Authors
Chen Y, Wang M, Romaguera J
  Title
Current regimens and novel agents for mantle cell lymphoma.
  Journal
Br J Haematol 167:3-18 (2014)
DOI:10.1111/bjh.13000
Reference
(drug)
  Authors
Papadakis MA and McPhee SJ |(ed)
  Title
Current Medical Diagnosis & Treatment 2015, Fifty-Fourth Edition
  Journal
McGraw Hill Education
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