| Entry |
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| Name |
Lymphoplasmacytic lymphoma
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| 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.
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| Category |
Cancer
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| Pathway |
| Transcriptional misregulation in cancers |
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| Gene |
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| Carcinogen |
Hepatitis C virus [VGNM: NC_004102]
*Azathioprine [DR: D00238]
*Ethylene oxide [CPD: C06548]
*Human immunodeficiency virus type 1 (infection with) [VGNM: NC_001802]
*2,3,7,8-Tetrachlorodibenzo-para-dioxin [CPD: C07557]
*: Although this is a risk factor for non-Hodgkin lymphoma in general, the strength of association with different histological tumor types is not well understood.
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| Marker |
IgM
CD19 [HSA: 930]
CD20 [HSA: 931]
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| Drug |
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| Comment |
ICD-O: 9671/3, Tumor type: Lymphoplasmacytic lymphoma
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| Other DBs |
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| Reference |
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| Authors |
Kristinsson SY, Koshiol J, Goldin LR, Bjorkholm M, Turesson I, Gridley G, McMaster ML, Landgren O |
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| Title |
Genetics- and immune-related factors in the pathogenesis of lymphoplasmacytic lymphoma/ Waldenstrom's macroglobulinemia. |
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| Journal |
Clin Lymphoma Myeloma 9:23-6 (2009) |
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| Reference |
PMID: 18499469 (description, gene, carcinogen) |
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| Authors |
Vitolo U, Ferreri AJ, Montoto S |
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| Title |
Lymphoplasmacytic lymphoma-Waldenstrom's macroglobulinemia. |
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| Journal |
Crit Rev Oncol Hematol 67:172-85 (2008) |
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| Reference |
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| Authors |
Arber DA. |
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| Title |
Molecular diagnostic approach to non-Hodgkin's lymphoma. |
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| Journal |
J Mol Diagn 2:178-90 (2000) |
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| Reference |
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| Authors |
Kuppers R. |
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| Title |
Mechanisms of B-cell lymphoma pathogenesis. |
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| Journal |
Nat Rev Cancer 5:251-62 (2005) |
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| Reference |
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| Authors |
Iida S, Rao PH, Ueda R, Chaganti RS, Dalla-Favera R |
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| Title |
Chromosomal rearrangement of the PAX-5 locus in lymphoplasmacytic lymphoma with t(9;14)(p13;q32). |
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| Journal |
Leuk Lymphoma 34:25-33 (1999) |
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| Reference |
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| Authors |
Ohno H, Ueda C, Akasaka T |
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| Title |
The t(9;14)(p13;q32) translocation in B-cell non-Hodgkin's lymphoma. |
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| Journal |
Leuk Lymphoma 36:435-45 (2000) |
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| Reference |
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| Authors |
Patel P, Moazzam N, Potti A, Mehdi SA. |
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| Title |
Azathioprine induced hodgkin lymphoma: a case report and review of literature. |
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| Journal |
Am J Clin Oncol 28:427-8 (2005) |
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| Reference |
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| Authors |
Kolman A, Chovanec M, Osterman-Golkar S. |
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| Title |
Genotoxic effects of ethylene oxide, propylene oxide and epichlorohydrin in humans: update review (1990-2001). |
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| Journal |
Mutat Res 512:173-94 (2002) |
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| Reference |
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| Authors |
Schottenfeld D, Beebe-Dimmer JL. |
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| Title |
Advances in cancer epidemiology: understanding causal mechanisms and the evidence for implementing interventions. |
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| Journal |
Annu Rev Public Health 26:37-60 (2005) |
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| Reference |
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| Authors |
Abnet CC. |
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| Title |
Carcinogenic food contaminants. |
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| Journal |
Cancer Invest 25:189-96 (2007) |
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| Reference |
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| Authors |
Konoplev S, Medeiros LJ, Bueso-Ramos CE, Jorgensen JL, Lin P. |
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| Title |
Immunophenotypic profile of lymphoplasmacytic lymphoma/Waldenstrom macroglobulinemia. |
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| Journal |
Am J Clin Pathol 124:414-20 (2005) |
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| Reference |
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| Authors |
Owen RG, Treon SP, Al-Katib A, Fonseca R, Greipp PR, McMaster ML, Morra E, Pangalis GA, San Miguel JF, Branagan AR, Dimopoulos MA. |
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| Title |
Clinicopathological definition of Waldenstrom's macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia. |
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| Journal |
Semin Oncol 30:110-5 (2003) |
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| Reference |
(drug) |
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| Authors |
McPhee SJ, Papadakis MA, Tierney LM (ed). |
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| Title |
Current Medical Diagnosis & Treatment 2007, Forty-Sixth Edition |
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| Journal |
The McGraw-Hill Companies, Inc. (2007) |
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