Literature DB >> 25800543

Second cancers and Richter transformation are the leading causes of death in patients with trisomy 12 chronic lymphocytic leukemia.

Paolo Strati1, Lynne V Abruzzo2, William G Wierda1, Susan O'Brien1, Alessandra Ferrajoli1, Michael J Keating3.   

Abstract

BACKGROUND: Trisomy 12 (+12) is detected by fluorescence in-situ hybridization (FISH) analysis in up to 20% of patients with chronic lymphocytic leukemia (CLL). Patients with +12 are known to have unique features and to carry an intermediate prognosis. PATIENTS AND METHODS: In order to better define this large group, we reviewed the characteristics of 250 untreated patients with +12.
RESULTS: When compared to 516 untreated patients negative for +12 by FISH, patients with +12 showed a higher incidence of thrombocytopenia, Richter transformation, and second malignant neoplasms (SMN), in addition to the expected increased rate of CD38 positivity and atypical immunophenotype. At a median follow-up of 51 months, 57% of patients needed first-line treatment; median time to first treatment was 38 months, and on multivariate analysis (MVA), it was found to be shorter in patients with advanced Rai stage, palpable splenomegaly, and deletion of 14q by conventional cytogenetic analysis. The overall response rate with first-line treatment was 94%. The median failure-free survival has not been reached, but on MVA, it was found to be shorter in patients whose disease responded in a manner other than complete remission or with FISH negativity for deletion 13q. The median overall survival for the entire group has not been reached, but MVA revealed it to be shorter in patients with an absolute lymphocyte count of > 30 × 10(9)/L or who developed SMN. Eighteen deaths have been observed so far, and Richter transformation and SMN were the leading causes of death (3 and 6, respectively).
CONCLUSION: Patients with +12 CLL show characteristic clinical and biologic features, and may benefit from increased surveillance for second cancers.
Copyright © 2015 Elsevier Inc. All rights reserved.

Entities:  

Keywords:  CLL; Prognosis; Richter transformation; Second cancers; Trisomy 12

Mesh:

Substances:

Year:  2015        PMID: 25800543      PMCID: PMC4874253          DOI: 10.1016/j.clml.2015.02.001

Source DB:  PubMed          Journal:  Clin Lymphoma Myeloma Leuk        ISSN: 2152-2669


  45 in total

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8.  Clinical, immunophenotypic, and molecular profiling of trisomy 12 in chronic lymphocytic leukemia and comparison with other karyotypic subgroups defined by cytogenetic analysis.

Authors:  Anastasia Athanasiadou; Kostas Stamatopoulos; Aliki Tsompanakou; Maria Gaitatzi; Panagiotis Kalogiannidis; Achilles Anagnostopoulos; Athanasios Fassas; A Tsezou
Journal:  Cancer Genet Cytogenet       Date:  2006-07-15

9.  Overall and cancer-specific survival of patients with breast, colon, kidney, and lung cancers with and without chronic lymphocytic leukemia: a SEER population-based study.

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10.  14q deletions are associated with trisomy 12, NOTCH1 mutations and unmutated IGHV genes in chronic lymphocytic leukemia and small lymphocytic lymphoma.

Authors:  Adrien Cosson; Elise Chapiro; Nabila Belhouachi; Hong-Anh Cung; Boris Keren; Frederik Damm; Caroline Algrin; Christine Lefebvre; Sandra Fert-Ferrer; Isabelle Luquet; Nathalie Gachard; Francine Mugneret; Christine Terre; Marie-Agnes Collonge-Rame; Lucienne Michaux; Isabelle Rafdord-Weiss; Pascaline Talmant; Lauren Veronese; Nathalie Nadal; Stephanie Struski; Carole Barin; Catherine Helias; Marina Lafage; Eric Lippert; Nathalie Auger; Virginie Eclache; Damien Roos-Weil; Veronique Leblond; Catherine Settegrana; Karim Maloum; Frederic Davi; Helene Merle-Beral; Claude Lesty; Florence Nguyen-Khac
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Journal:  Nat Rev Cancer       Date:  2016-03       Impact factor: 60.716

2.  Mutational status of IGHV is the most reliable prognostic marker in trisomy 12 chronic lymphocytic leukemia.

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3.  Incidental Richter transformation in chronic lymphocytic leukemia patients during temporary interruption of ibrutinib.

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5.  Trisomy 12 chronic lymphocytic leukemia expresses a unique set of activated and targetable pathways.

Authors:  Lynne V Abruzzo; Carmen D Herling; George A Calin; Christopher Oakes; Lynn L Barron; Haley E Banks; Vikram Katju; Michael J Keating; Kevin R Coombes
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Review 6.  Pathophysiology of chronic lymphocytic leukemia and human B1 cell development.

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7.  [The relationship between NOTCH1 mutation and the Richter transformation in chronic lymphocytic leukemia].

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Journal:  Zhonghua Xue Ye Xue Za Zhi       Date:  2018-09-14

Review 8.  Morphological, immunophenotypic, and genetic features of chronic lymphocytic leukemia with trisomy 12: a comprehensive review.

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