Biclonal lymphoproliferative disorders: another association with NOTCH1-mutated chronic lymphocytic leukaemias.

Abstract:

INTRODUCTION:Biclonal lymphoid disorders, when two distinct lymphoproliferative disorders (LPD) co-exist, are rare (incidence of 1.4%) and associated with a poor prognosis. NOTCH1 mutations occur in 10% of CLL at diagnosis, associated with a short disease-free interval and increased risk of Richter's transformation. We hypothesised that the incidence of NOTCH1 mutations in CLL with a second LPD may be increased, because the mutation occurs early in leukaemogenesis, permitting clonal divergence. METHODS:We identified 19 patients with biclonal LPD at diagnosis: 11 with CLL and a second LPD (group A) and 8 with a second distinct CLL (group B). NOTCH1 mutation analysis was performed and clinical outcome investigated. RESULTS:Ten of 19 (52%) were NOTCH1 mutated: 5 in group A (45%) and 5 in group B (62.5%) with a favourable clinical outcome observed among this cohort with 28.7 (range 1-99) months of follow-up. CONCLUSION:In conclusion, we identified a significant (52%) incidence of NOTCH1 mutations in CLL in the context of biclonal LPD, associated with an indolent clinical course.

journal_name

Ir J Med Sci

authors

Fogarty H,Dowling A,O'Brien D,Langabeer S,Bacon CL,Flavin R,O'Dwyer M,Hennessy B,O'Leary H,Crotty G,Henderson R,Nolan J,Thornton P,Vandenberghe E,Quinn F

doi

10.1007/s11845-020-02386-1

subject

Has Abstract

pub_date

2020-10-17 00:00:00

eissn

0021-1265

issn

1863-4362

pii

10.1007/s11845-020-02386-1

pub_type

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