Waldenstrom's macroglobulinemia (WM)

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Waldenstrom's macroglobulinemia (WM)

Clinics and Pathology

Disease Waldenstrom's macroglobulinemia (corresponding to lymphoplasmacytoid lymphoma / Immunocytoma in the REAL and WHO classification)

Phenotype / cell stem origin Stem cell origin: Post-germinal centre IgM-bearing memory B-cell. Phenotype: CD19+; CD20+; CD22+; FMC7+; CD38+; cytoplasmic IgM bright+; CD5-; CD23-; CD10-.

Epidemiology It accounts for 1-2% of all nodal lymphomas. The annual incidence falls in the 0,2-0,6% range per 100 000.

Clinics Indolent lymphoma characterized by the secretion of a monoclonal IgM protein and by the expansion of the neoplastic clone in the bone marrow, in the lymphoid tissue and in extra-nodal sites. The symptoms derive from tissue infiltration, bone marrow failure and from the presence of the IgM paraprotein (hyperviscosity syndrome, polyneuropathies, AL amyloidosis)

Pathology Diffuse proliferation of small lymphocytes, plasmacytoid lymphocytes and plasma cells, infiltrating the bone marrow and the interfollicular areas of the lymph nodes. By definition, this lymphoma subtype lacks the diagnostic features chronic lymphocytic leukemia (CLL), mantle cell lymphoma and follicle centre cell lymphoma. Transformation into high grade lymphoma may occur in a minority (5%) of cases..

Treatment Alkylating agents (chlorambucil), multiagent chemotherapy (vincristine cyclophosphamide, steroid, with or without anthtacyclines); fludarabine, with or without mitoxantrone.

Prognosis Median survival exceeds 5 years and there is no plateau in the survival curve, just as in other low-grade lymphomas.

Cytogenetics

Cytogenetics Morphological There is no chromosome anomaly that is specific for WM. The overall cytogenetic picture is similar to that of marginal zone B-cell lymphoma. Recurrent structural changes include

the t(9;14)(p13;q32)
the t(11;18)(q21;q21)
a 6q- chromosome
Numerical abberrations involving several chromosomes were also detected. Recurrent aberrations include trisomy 1q, and trisomy 3q

Genes involved and Proteins

Note The t(9;14)(p13;q32), fuses PAX5 with the IgH gene. PAX5 was shown to code for the BSAP (B-cell specific activator protein). The t(11;18)(q21;q21) is cytogenetically indistinguishable from the classical t(11;18) found in MALT lymphoma, where involvement of the API2- MLT genes was demonstrated.

Bibliography

t(9;14)(p13;q32) denotes a subset of low-grade non-Hodgkin's lymphoma with plasmacytoid differentiation.

Offit K, Parsa NZ, Filippa D, Jhanwar SC, Chaganti RS

Blood. 1992 ; 80 (10) : 2594-2599.

PMID 1384792

A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group.

Harris NL, Jaffe ES, Stein H, Banks PM, Chan JK, Cleary ML, Delsol G, De Wolf-Peeters C, Falini B, Gatter KC

Blood. 1994 ; 84 (5) : 1361-1392.

PMID 8068936

World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues: report of the Clinical Advisory Committee meeting-Airlie House, Virginia, November 1997.

Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J, Lister TA, Bloomfield CD

Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 1999 ; 17 (12) : 3835-3849.

PMID 10577857

Waldenstrom's macroglobulinemia. Clinical manifestations and prognosis.

Merlini G

Education Program Book, American Society of Hematology, New Orleans,..

Indolent lymphomas. Different entities and diagnostic problems.

Berger F

Educational book..

Contributor(s)

Written 01-2002 Antonio Cuneo and Gianluigi Castoldi

Citation

This paper should be referenced as such :
Cuneo A, Castoldi GL . Waldenstrom's macroglobulinemia (WM). Atlas Genet Cytogenet Oncol Haematol. January 2002 .
URL : http://AtlasGeneticsOncology.org/Genes/WaldensID2043.html

© Atlas of Genetics and Cytogenetics in Oncology and Haematology
indexed on : Sat Dec 6 18:01:40 2008

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Disease	Waldenstrom's macroglobulinemia (corresponding to lymphoplasmacytoid lymphoma / Immunocytoma in the REAL and WHO classification)
Phenotype / cell stem origin	Stem cell origin: Post-germinal centre IgM-bearing memory B-cell. Phenotype: CD19+; CD20+; CD22+; FMC7+; CD38+; cytoplasmic IgM bright+; CD5-; CD23-; CD10-.
Epidemiology	It accounts for 1-2% of all nodal lymphomas. The annual incidence falls in the 0,2-0,6% range per 100 000.
Clinics	Indolent lymphoma characterized by the secretion of a monoclonal IgM protein and by the expansion of the neoplastic clone in the bone marrow, in the lymphoid tissue and in extra-nodal sites. The symptoms derive from tissue infiltration, bone marrow failure and from the presence of the IgM paraprotein (hyperviscosity syndrome, polyneuropathies, AL amyloidosis)
Pathology	Diffuse proliferation of small lymphocytes, plasmacytoid lymphocytes and plasma cells, infiltrating the bone marrow and the interfollicular areas of the lymph nodes. By definition, this lymphoma subtype lacks the diagnostic features chronic lymphocytic leukemia (CLL), mantle cell lymphoma and follicle centre cell lymphoma. Transformation into high grade lymphoma may occur in a minority (5%) of cases..
Treatment	Alkylating agents (chlorambucil), multiagent chemotherapy (vincristine cyclophosphamide, steroid, with or without anthtacyclines); fludarabine, with or without mitoxantrone.
Prognosis	Median survival exceeds 5 years and there is no plateau in the survival curve, just as in other low-grade lymphomas.