Scientific publications

Origin of Waldenstrom's macroglobulinaemia

Jun 1, 2016 | Magazine: Best Practice & Research. Clinical Haematology

Ramón García-Sanz  1 , Cristina Jiménez  2 , Noemí Puig  2 , Bruno Paiva  3 , Norma C Gutiérrez  2 , Paula Rodríguez-Otero  3 , Julia Almeida  4 , Jesús San Miguel  3 , Alberto Orfão  4 , Marcos González  2 , Martín Pérez-Andrés  4


Waldenstrom's macroglobulinaemia (WM) is an MYD88L265P-mutated lymphoplasmacytic lymphoma that invades bone marrow and secretes monoclonal immunoglobulin M (IgM). WM cells are usually unable to undergo class switch recombination, and have mutated IGHV, with a typical immunophenotype CD19+/CD22low+/CD23-/CD25+/CD27+/CD45+/CD38low+/SmIgM+ (negative for CD5, CD10, CD11c, CD103).

This immunophenotype matches memory B cells (smIgM-/+/CD10-/CD19+/CD20+/CD27+/CD38low+/CD45+), representing 30% of B cells in the blood. Fifty percent of them have not undergone class switch recombination and are IgM+.

These cells have suffered somatic hypermutation as WM cells. Genetic abnormalities do not abrogate the capacity to progress to plasma cells that usually belong to the clonal WM compartment, with a normal immunophenotype and functional characteristics.

However, some WM cells are CD27-, MYD88WT, without somatic hypermutation, or with class switch recombination capable of reactivation. Thus, most data support a B-memory-cell origin for WM, but a small fraction of cases may have a different origin.

CITATION  Best Pract Res Clin Haematol. 2016 Jun;29(2):136-147. doi: 10.1016/j.beha.2016.08.024. Epub 2016 Oct 6. 

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