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Vol 55, No 3 (2017)
Review paper
Submitted: 2017-07-25
Accepted: 2017-10-04
Published online: 2017-10-09
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Novel prognostic molecular factors: a quantum leap in the field of chronic lymphocytic leukemia

Ewelina Zakrzewska1, Marta Pirog1, Joanna Purkot1, Krzysztof Giannopoulos12
·
Pubmed: 28994094
·
Folia Histochem Cytobiol 2017;55(3):95-106.
Affiliations
  1. Department of Experimental Hematooncology, Medical University of Lublin, Poland
  2. Department of Hematology, St John’s Cancer Center, Lublin, Poland

open access

Vol 55, No 3 (2017)
REVIEW
Submitted: 2017-07-25
Accepted: 2017-10-04
Published online: 2017-10-09

Abstract

Cytogenetic lesions do not completely explain clinical heterogeneity of chronic lymphocytic leukemia (CLL). The 2016 revision of the World Health Organization classification 2008 indicated that molecular lesions of TP53, NOTCH1, SF3B1 and BIRC3 have potential clinical relevance and could be integrated into an updated risk profile. The negative clinical implications of TP53 disruptions are well constituted and patients with these mutations should be considered for novel, small molecule signal transduction inhibitors therapies. Mutations of NOTCH1, SF3B1 and BIRC3 are associated with poor prognosis. Patients with mutated SF3B1 or NOTCH1 genes present shorter time to first treatment compared to unmutated group. NOTCH1 mutations are related to a high risk of Richter’s syndrome transformation, especially in case of TP53 disruptions’ coexistence. Large studies on MYD88 mutations in CLL have not explained clearly their clinical importance.The aim of this paper is to provide a comprehensive review on novel molecular aberrations identified in CLL.

Abstract

Cytogenetic lesions do not completely explain clinical heterogeneity of chronic lymphocytic leukemia (CLL). The 2016 revision of the World Health Organization classification 2008 indicated that molecular lesions of TP53, NOTCH1, SF3B1 and BIRC3 have potential clinical relevance and could be integrated into an updated risk profile. The negative clinical implications of TP53 disruptions are well constituted and patients with these mutations should be considered for novel, small molecule signal transduction inhibitors therapies. Mutations of NOTCH1, SF3B1 and BIRC3 are associated with poor prognosis. Patients with mutated SF3B1 or NOTCH1 genes present shorter time to first treatment compared to unmutated group. NOTCH1 mutations are related to a high risk of Richter’s syndrome transformation, especially in case of TP53 disruptions’ coexistence. Large studies on MYD88 mutations in CLL have not explained clearly their clinical importance.The aim of this paper is to provide a comprehensive review on novel molecular aberrations identified in CLL.

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Keywords

chronic lymphocytic leukemia; TP53; NOTCH1; SF3B1; BIRC3; MYD88; prognostic factors

About this article
Title

Novel prognostic molecular factors: a quantum leap in the field of chronic lymphocytic leukemia

Journal

Folia Histochemica et Cytobiologica

Issue

Vol 55, No 3 (2017)

Article type

Review paper

Pages

95-106

Published online

2017-10-09

Page views

1892

Article views/downloads

1821

DOI

10.5603/FHC.a2017.0019

Pubmed

28994094

Bibliographic record

Folia Histochem Cytobiol 2017;55(3):95-106.

Keywords

chronic lymphocytic leukemia
TP53
NOTCH1
SF3B1
BIRC3
MYD88
prognostic factors

Authors

Ewelina Zakrzewska
Marta Pirog
Joanna Purkot
Krzysztof Giannopoulos

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