4.6 Article

6q deletion in Waldenstrom macroglobulinaemia negatively affects time to transformation and survival

Journal

BRITISH JOURNAL OF HAEMATOLOGY
Volume 192, Issue 5, Pages 843-852

Publisher

WILEY
DOI: 10.1111/bjh.17028

Keywords

6q deletion; Waldenstrom macroglobulinaemia; IgM-MGUS; FISH; prognosis

Categories

Funding

  1. SIE (Societa Italiana di Ematologia)
  2. AIL (Associazione Italiana contro le Leucemie, Linfomi e Mieloma)
  3. ISCIII [CD19/00030, CP13/00080]
  4. Fundacion Espanola de Hematologia y Hemoterapia (FEHH)
  5. Asociacion Espanola Contra El Cancer (AECC)

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The presence of del6q in IgM gammopathy is associated with symptomatic disease, need for treatment and poorer clinical outcomes.
Deletion of the long arm of chromosome 6 (del6q) is the most frequent cytogenetic abnormality in Waldenstrom macroglobulinaemia (WM), occurring in approximately 50% of patients. Its effect on patient outcome has not been completely established. We used fluorescencein situhybridisation to analyse the prevalence of del6q in selected CD19+ bone marrow cells of 225 patients with newly diagnosed immunoglobulin M (IgM) monoclonal gammopathies. Del6q was identified in one of 27 (4%) cases of IgM-monoclonal gammopathy of undetermined significance, nine of 105 (9%) of asymptomatic WM (aWM), and 28/93 (30%) of symptomatic WM (sWM), and was associated with adverse prognostic features and higher International Prognostic Scoring System for WM (IPSSWM) score. Asymptomatic patients with del6q ultimately required therapy more often and had a shorter time to transformation (TT) to symptomatic disease (median TT, 30 months vs. 199 months, respectively,P < 0 center dot 001). When treatment was required, 6q-deleted patients had shorter progression-free survival (median 20 vs. 47 months,P < 0 center dot 001). The presence of del6q translated into shorter overall survival (OS), irrespective of the initial diagnosis, with a median OS of 90 compared with 131 months in non-del6q patients (P = 0 center dot 01). In summary, our study shows that del6q in IgM gammopathy is associated with symptomatic disease, need for treatment and poorer clinical outcomes.

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