KEGG   DISEASE: Lymphoplasmacytic lymphomaHelp
Entry
H00011                      Disease                                

Name
Lymphoplasmacytic lymphoma
Description
Lymphoplasmacytic lymphoma (LPL)/Waldenstrom's macroglobulinemia (WM) is a non-Hodgkin lymphoma (NHL) subtype characterized by small B-lymphocytes, plasmacytoid lymphocytes, and plasma cells, usually involving bone marrow, lymph nodes, and the spleen. WM can be distinguished clinically from LPL on the basis of a detectable monoclonal (IgM) immunoglobulin spike in serum. In the general population, LPL/WM is a very rare disease, accounting for only 1%-2% of all hematologic malignancies, reflected in an incidence rate of 3-4 cases per million people per year. The t(9;14)(p13;q32) is present in near 50% of cases of LPL. This chromosomal translocation involves a junction between 9p13 and the switch micro region of the Ig heavy chain locus on 14q32. The 9p13 breakpoint contains the PAX-5 gene which encodes a B-cell specific transcription factor involved in the control of B-cell proliferation and differentiation. The translocation causes the juxtaposition of the PAX-5 gene to the IgH locus in the opposite direction of transcription, resulting in an 11-fold over-expression of PAX-5 mRNA.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of haematopoietic and lymphoid tissues
   H00011  Lymphoplasmacytic 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
     H00011  Lymphoplasmacytic lymphoma
Tumor markers [br08442.html]
 H00011
BRITE hierarchy
Pathway
hsa05202  Transcriptional misregulation in cancer
Gene
Pax5-IgH (translocation) [HSA:5079] [KO:K09383]
Carcinogen
Azathioprine [DG:DG00743]
Ethylene oxide [CPD:C06548]
2,3,7,8-Tetrachlorodibenzo-para-dioxin [CPD:C07557]
Pathogen
Hepatitis C virus [GN:T40066]
Human immunodeficiency virus 1 (HIV-1) [GN:T40001]
Drug
Carmustine [DR:D00254]
Doxorubicin hydrochloride [DR:D01275]
Rituximab [DR:D02994] (CD20-positive)
Ibrutinib [DR:D10223]
Plerixafor [DR:D08971]
Ibritumomab tiuxetan [DR:D04489]
Comment
ICD-O: 9671/3, Tumor type: Lymphoplasmacytic lymphoma
Although the carcinogens listed and infection with HIV-1 are risk factors for non-Hodgkin lymphoma in general, the strength of association with different histological tumor types is not well understood.
Other DBs
ICD-11: 2A85.4
ICD-10: C83.8 C88.0
Reference
  Authors
Kristinsson SY, Koshiol J, Goldin LR, Bjorkholm M, Turesson I, Gridley G, McMaster ML, Landgren O
  Title
Genetics- and immune-related factors in the pathogenesis of lymphoplasmacytic lymphoma/ Waldenstrom's macroglobulinemia.
  Journal
Clin Lymphoma Myeloma 9:23-6 (2009)
DOI:10.3816/CLM.2009.n.004
Reference
PMID:18499469 (description, gene, carcinogen)
  Authors
Vitolo U, Ferreri AJ, Montoto S
  Title
Lymphoplasmacytic lymphoma-Waldenstrom's macroglobulinemia.
  Journal
Crit Rev Oncol Hematol 67:172-85 (2008)
DOI:10.1016/j.critrevonc.2008.03.008
Reference
PMID:11232108 (gene, tumor type)
  Authors
Arber DA.
  Title
Molecular diagnostic approach to non-Hodgkin's lymphoma.
  Journal
J Mol Diagn 2:178-90 (2000)
DOI:10.1016/S1525-1578(10)60636-8
Reference
  Authors
Kuppers R.
  Title
Mechanisms of B-cell lymphoma pathogenesis.
  Journal
Nat Rev Cancer 5:251-62 (2005)
DOI:10.1038/nrc1589
Reference
  Authors
Iida S, Rao PH, Ueda R, Chaganti RS, Dalla-Favera R
  Title
Chromosomal rearrangement of the PAX-5 locus in lymphoplasmacytic lymphoma with t(9;14)(p13;q32).
  Journal
Leuk Lymphoma 34:25-33 (1999)
DOI:10.3109/10428199909083377
Reference
  Authors
Ohno H, Ueda C, Akasaka T
  Title
The t(9;14)(p13;q32) translocation in B-cell non-Hodgkin's lymphoma.
  Journal
Leuk Lymphoma 36:435-45 (2000)
DOI:10.3109/10428190009148390
Reference
PMID:16062089 (carcinogen)
  Authors
Patel P, Moazzam N, Potti A, Mehdi SA.
  Title
Azathioprine induced hodgkin lymphoma: a case report and review of literature.
  Journal
Am J Clin Oncol 28:427-8 (2005)
DOI:10.1097/01.coc.0000168670.28659.8d
Reference
PMID:12464351 (carcinogen)
  Authors
Kolman A, Chovanec M, Osterman-Golkar S.
  Title
Genotoxic effects of ethylene oxide, propylene oxide and epichlorohydrin in humans: update review (1990-2001).
  Journal
Mutat Res 512:173-94 (2002)
DOI:10.1016/S1383-5742(02)00067-4
Reference
PMID:15760280 (carcinogen)
  Authors
Schottenfeld D, Beebe-Dimmer JL.
  Title
Advances in cancer epidemiology: understanding causal mechanisms and the evidence for implementing interventions.
  Journal
Annu Rev Public Health 26:37-60 (2005)
DOI:10.1146/annurev.publhealth.26.021304.144402
Reference
PMID:17530489 (carcinogen)
  Authors
Abnet CC.
  Title
Carcinogenic food contaminants.
  Journal
Cancer Invest 25:189-96 (2007)
DOI:10.1080/07357900701208733

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