Lymphoplasmacytic lymphoma

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.

Cancer

Pax5-IgH (translocation) [HSA:5079] [KO:K09383]

Azathioprine [CPD:C06837]
Ethylene oxide [CPD:C06548]
2,3,7,8-Tetrachlorodibenzo-para-dioxin [CPD:C07557]

Hepatitis C virus [GN:T40066]
Human immunodeficiency virus 1 (HIV-1) [GN:T40001]

Carmustine [DR:D00254]
Doxorubicin hydrochloride [DR:D01275]
Rituximab [DR:D02994] (CD20-positive)
Ibrutinib [DR:D10223]
Plerixafor [DR:D08971]
Ibritumomab tiuxetan [DR:D04489]

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.

PMID:19362964

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PMID:15803153

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PMID:10350329

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PMID:10784387

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PMID:16062089 (carcinogen)

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PMID:12464351 (carcinogen)

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Mutat Res 512:173-94 (2002)
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PMID:15760280 (carcinogen)

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PMID:17530489 (carcinogen)

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Carcinogenic food contaminants.

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