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Trisomy 4, a new chromosomal abnormality in Waldenström's macroglobulinemia: a study of 39 cases

Leukemia volume 20, pages 1634–1636 (2006)Cite this article

Waldenström's macroglobulinemia (WM) is a rare B-cell disorder characterized by the accumulation of monoclonal lymphoplasmocytic cells in bone marrow in association with a serum monoclonal immunoglobulin (Ig)M protein. Patients may present with organomegaly, anemia, hyperviscosity, cryoglobulinemia, amyloidosis and autoimmune disease.

The genetic basis of this disorder is poorly understood. Cytogenetic abnormalities differ from those commonly reported in other B-cell neoplasms. Translocations involving immunoglobulin heavy-chain gene (IgH) are a common genetic change in multiple myeloma (MM) (75%) and a recurrent abnormality in many B-cell lymphomas such as t(11;14)(q123;q32) in mantle cell lymphoma, t(14;18)(q32;q21) in follicular lymphoma and t(9;14)(p13;q32) in lymphoplasmocytic lymphoma. On the other hand, the incidence of IgH rearrangements is low in WM (<3%).1, 2 The translocation t(11;18)(q21;q21), a specific abnormality, observed in about 20% of extranodal marginal zone lymphomas has been reported in few cases of patients with WM.3, 4 However, this translocation was not detected in the tumor cells of 24 WM patients using well-validated probe for its detection.1 Deletions of chromosome 13q14 are present in approximately 50% of patients with MM or B-cell chronic lymphocytic leukemia (B-CLL), but are rare in WM at the time of diagnosis (<5%) and with a low frequency in patients after treatment (15%).5 The deletion of the long arm of chromosome 6 is a recurrent abnormality reported in about 50%, in cases of WM mainly in advanced disease and is frequently associated with clonal evolutions (1Schop et al. Blood, 2005; 106: 290; abstract).

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References

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Authors and Affiliations

  1. Cytogenetic Laboratory, Mignot Hospital, Versailles, France

    C Terré

  2. Cytogenetic Laboratory, Pitie Salpetriere Hospital AP-HP, Paris, France

    F Nguyen-Khac & E Chapiro

  3. Cytogenetic Laboratory, Bretonneau Hospital, Tours, France

    C Barin

  4. Biopathology Laboratory, Paoli-Calmettes Institute, Marseille, France

    M J Mozziconacci

  5. Cytogenetic Laboratory, Avicenne Hospital AP-HP, Bobigny, France

    V Eclache

  6. Cytogenetic Laboratory, Kremlin-Bicêtre Hospital AP-HP, Paris, France

    C Léonard

  7. Hematology Department, Mignot Hospital, Versailles, France

    H Farhat, P Rousselot & S Castaigne

  8. Hematology Department, Pitie Salpetriere Hospital AP-HP, Paris, France

    A Bouyon, S Choquet & V Leblond

  9. Biochemestry Laboratory, Mignot Hospital, Versailles, France

    M Spentchian

  10. UMR 599 Inserm, Paoli-Calmettes Institute, Marseille, France

    P Dubreuil

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  1. C Terré
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Correspondence to C Terré.

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Terré, C., Nguyen-Khac, F., Barin, C. et al. Trisomy 4, a new chromosomal abnormality in Waldenström's macroglobulinemia: a study of 39 cases. Leukemia 20, 1634–1636 (2006). https://doi.org/10.1038/sj.leu.2404314

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