KEGG   DISEASE: Multiple myelomaHelp
Entry
H00010                      Disease                                

Name
Multiple myeloma
Description
Multiple myeloma is a disorder in which malignant plasma cells accumulate, generally derived from one clone in the bone marrow. Intricate interactions occur between the bone-marrow microenvironment and the myeloma cells, frequently causing bone destruction, which in turn stimulates tumor growth. Often it is preceded by a premalignant tumor called monoclonal gammopathy of undetermined significance (MGUS). Multiple oncogenic events have been identified that have contributed to the pathogenesis of myeloma. Among the earliest genetic events are translocations of the immunoglobulin heavy-chain gene locus, which leads to dysregulation of oncogenes at translocation partner regions (cyclin D1 at 11q13, FGFR3/MMSET at 4p16.3, c-MAF at 16q23, and cyclin D3 at 6p21), and deletions of 13q14, the site of a putative tumor suppressor gene. Additional molecular events include epigenetic changes and activation of oncogenes (mutations of N-RAS and K-RAS, and changes in c-MYC), which are usually associated with disease progression.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of haematopoietic and lymphoid tissues
   H00010  Multiple myeloma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Neoplasms of haematopoietic or lymphoid tissues
   Mature B-cell neoplasms
    2A83  Plasma cell neoplasms
     H00010  Multiple myeloma
Tumor markers [br08442.html]
 H00010
BRITE hierarchy
Pathway
hsa05202  Transcriptional misregulation in cancer
Network
N00124  IGH-MAF fusion to transcriptional activation
N00125  IGH-MMSET fusion to transcriptional activation
Gene
CCND1-IgH (translocation) [HSA:595] [KO:K04503]
CCND3-IgH (translocation) [HSA:896] [KO:K10152]
IgH-FGFR3 (translocation) [HSA:2261] [KO:K05094]
IgH-MMSET (translocation) [HSA:7468] [KO:K11424]
IgH-MAF (translocation) [HSA:4094] [KO:K09035]
N-ras (activating mutation) [HSA:4893] [KO:K07828]
K-ras (activating mutation) [HSA:3845] [KO:K07827]
c-MYC (dysregulation) [HSA:4609] [KO:K04377]
p53 (mutation) [HSA:7157] [KO:K04451]
Drug
Cyclophosphamide [DR:D00287]
Melphalan [DR:D00369]
Melphalan hydrochloride [DR:D08173]
Carmustine [DR:D00254]
Doxorubicin hydrochloride [DR:D01275]
Elotuzumab [DR:D09337]
Daratumumab [DR:D10777]
Bortezomib [DR:D03150]
Panobinostat lactate [DR:D10019]
Carfilzomib [DR:D08880]
Ixazomib citrate [DR:D10131]
Plerixafor [DR:D08971]
Thalidomide [DR:D00754]
Lenalidomide [DR:D04687]
Pomalidomide [DR:D08976]
Comment
ICD-O: 9732/3, Tumor type: Plasma cell myeloma
Other DBs
ICD-11: 2A83.1
ICD-10: C90.0
MeSH: D009101
Reference
  Authors
Sirohi B, Powles R
  Title
Multiple myeloma.
  Journal
Lancet 363:875-87 (2004)
DOI:10.1016/S0140-6736(04)15736-X
Reference
  Authors
Seidl S, Kaufmann H, Drach J
  Title
New insights into the pathophysiology of multiple myeloma.
  Journal
Lancet Oncol 4:557-64 (2003)
DOI:10.1016/S1470-2045(03)01195-1
Reference
  Authors
Joy Ho P
  Title
Chromosomal and genetic abnormalities in myeloma.
  Journal
Clin Lab Haematol 24:259-69 (2002)
DOI:10.1046/j.1365-2257.2002.00456.x
Reference
PMID:16155016 (gene, tumor type)
  Authors
Bergsagel PL, Kuehl WM.
  Title
Molecular pathogenesis and a consequent classification of multiple myeloma.
  Journal
J Clin Oncol 23:6333-8 (2005)
DOI:10.1200/JCO.2005.05.021
Reference
  Authors
Podczaski E, Cain J.
  Title
Multiple myeloma.
  Journal
Clin Obstet Gynecol 45:928-38 (2002)
Reference
PMID:9414264
  Authors
Hallek M, Bergsagel PL, Anderson KC.
  Title
Multiple myeloma: increasing evidence for a multistep transformation process.
  Journal
Blood 91:3-21 (1998)
Reference
  Authors
Bergsagel PL, Kuehl WM
  Title
Chromosome translocations in multiple myeloma.
  Journal
Oncogene 20:5611-22 (2001)
DOI:10.1038/sj.onc.1204641

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