| H00011 | |
| H number | H00011 |
| Name | Lymphoplasmacytic lymphoma |
| Description | Lymphoplasmacytic lymphoma (LPL)/Waldenstrom's macroglobulinemia (WM) is a non-Hodgkin lymphoma (NHL) subtype. LPL/WM is a neoplasm of 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 IgM monoclonal 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 |
| Network | - |
| Gene | Pax5-IgH (translocation) [HSA:5079] [KO:K09383] |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | Ibrutinib [DR:D10223] Ibritumomab tiuxetan [DR:D04489] (CD20-directed) |
| Comment | - |
| Other DBs | ICD-11: 2A85.4 ICD-10: C83.8 C88.0 |
| Reference | PMID:19362964 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 PMID:18499469 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 PMID:11232108 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 PMID:15803153 AUTHORS Kuppers R. TITLE Mechanisms of B-cell lymphoma pathogenesis. JOURNAL Nat Rev Cancer 5:251-62 (2005) DOI:10.1038/nrc1589 PMID:10350329 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 PMID:10784387 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 PMID:16062089 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 PMID:12464351 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 PMID:15760280 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 PMID:17530489 AUTHORS Abnet CC. TITLE Carcinogenic food contaminants. JOURNAL Cancer Invest 25:189-96 (2007) DOI:10.1080/07357900701208733 |