Lymphoplasmacytic lymphoma

Non-Hodgkin lymphoma [DS:H02418]

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]

Ibrutinib [DR:D10223]
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

Kristinsson SY, Koshiol J, Goldin LR, Bjorkholm M, Turesson I, Gridley G, McMaster ML, Landgren O

Genetics- and immune-related factors in the pathogenesis of lymphoplasmacytic lymphoma/ Waldenstrom's macroglobulinemia.

Clin Lymphoma Myeloma 9:23-6 (2009)
DOI:10.3816/CLM.2009.n.004

PMID:18499469 (description, gene, carcinogen)

Vitolo U, Ferreri AJ, Montoto S

Lymphoplasmacytic lymphoma-Waldenstrom's macroglobulinemia.

Crit Rev Oncol Hematol 67:172-85 (2008)
DOI:10.1016/j.critrevonc.2008.03.008

PMID:11232108 (gene, tumor type)

Arber DA.

Molecular diagnostic approach to non-Hodgkin's lymphoma.

J Mol Diagn 2:178-90 (2000)
DOI:10.1016/S1525-1578(10)60636-8

PMID:15803153

Kuppers R.

Mechanisms of B-cell lymphoma pathogenesis.

Nat Rev Cancer 5:251-62 (2005)
DOI:10.1038/nrc1589

PMID:10350329

Iida S, Rao PH, Ueda R, Chaganti RS, Dalla-Favera R

Chromosomal rearrangement of the PAX-5 locus in lymphoplasmacytic lymphoma with t(9;14)(p13;q32).

Leuk Lymphoma 34:25-33 (1999)
DOI:10.3109/10428199909083377

PMID:10784387

Ohno H, Ueda C, Akasaka T

The t(9;14)(p13;q32) translocation in B-cell non-Hodgkin's lymphoma.

Leuk Lymphoma 36:435-45 (2000)
DOI:10.3109/10428190009148390

PMID:16062089 (carcinogen)

Patel P, Moazzam N, Potti A, Mehdi SA.

Azathioprine induced hodgkin lymphoma: a case report and review of literature.

Am J Clin Oncol 28:427-8 (2005)
DOI:10.1097/01.coc.0000168670.28659.8d

PMID:12464351 (carcinogen)

Kolman A, Chovanec M, Osterman-Golkar S.

Genotoxic effects of ethylene oxide, propylene oxide and epichlorohydrin in humans: update review (1990-2001).

Mutat Res 512:173-94 (2002)
DOI:10.1016/S1383-5742(02)00067-4

PMID:15760280 (carcinogen)

Schottenfeld D, Beebe-Dimmer JL.

Advances in cancer epidemiology: understanding causal mechanisms and the evidence for implementing interventions.

Annu Rev Public Health 26:37-60 (2005)
DOI:10.1146/annurev.publhealth.26.021304.144402

PMID:17530489 (carcinogen)

Abnet CC.

Carcinogenic food contaminants.

Cancer Invest 25:189-96 (2007)
DOI:10.1080/07357900701208733