Entry |
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Name |
Lymphoplasmacytic lymphoma |
Supergrp |
Non-Hodgkin lymphoma [DS: H02418] |
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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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
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Pathway |
hsa05202 | Transcriptional misregulation in cancer |
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Gene |
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Carcinogen |
Azathioprine [CPD: C06837]
Ethylene oxide [CPD: C06548]
2,3,7,8-Tetrachlorodibenzo-para-dioxin [CPD: C07557]
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Drug |
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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.
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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 |
Title |
Genetics- and immune-related factors in the pathogenesis of lymphoplasmacytic lymphoma/ Waldenstrom's macroglobulinemia. |
Journal |
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Reference |
PMID: 18499469 (description, gene, carcinogen) |
Authors |
Vitolo U, Ferreri AJ, Montoto S |
Title |
Lymphoplasmacytic lymphoma-Waldenstrom's macroglobulinemia. |
Journal |
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Reference |
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Authors |
Arber DA. |
Title |
Molecular diagnostic approach to non-Hodgkin's lymphoma. |
Journal |
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Reference |
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Authors |
Kuppers R. |
Title |
Mechanisms of B-cell lymphoma pathogenesis. |
Journal |
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Reference |
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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 |
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Reference |
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Authors |
Ohno H, Ueda C, Akasaka T |
Title |
The t(9;14)(p13;q32) translocation in B-cell non-Hodgkin's lymphoma. |
Journal |
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Reference |
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Authors |
Patel P, Moazzam N, Potti A, Mehdi SA. |
Title |
Azathioprine induced hodgkin lymphoma: a case report and review of literature. |
Journal |
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Reference |
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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 |
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Reference |
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Authors |
Schottenfeld D, Beebe-Dimmer JL. |
Title |
Advances in cancer epidemiology: understanding causal mechanisms and the evidence for implementing interventions. |
Journal |
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Reference |
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Authors |
Abnet CC. |
Title |
Carcinogenic food contaminants. |
Journal |
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LinkDB |
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