DISEASE: Lymphoplasmacytic lymphoma

Entry

H00011                      Disease

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.

Category

Cancer

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

Gene

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

Drug

Ibrutinib [DR:D10223]
Ibritumomab tiuxetan [DR:D04489]

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

Reference

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

Reference

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

Reference

PMID:15803153

Authors

Kuppers R.

Title

Mechanisms of B-cell lymphoma pathogenesis.

Journal

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

Reference

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

Reference

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

Reference

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

Reference

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

Reference

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

Reference

PMID:17530489

Authors

Abnet CC.

Title

Carcinogenic food contaminants.

Journal

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

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Ontology (1)   
   KEGG BRITE (1)   
Drug (34)   
   KEGG DRUG (34)   
Gene (2)   
   KEGG ORTHOLOGY (1)   
   KEGG GENES (1)   
Literature (10)   
   PubMed (10)   
All databases (47)   

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