Immunoglobulin genes in chronic lymphocytic leukemia: key to understanding the disease and improving risk stratification

SUTTON, LA., A. HADZIDIMITRIOU, P. BALIAKAS, A. AGATHANGELIDIS, A. LANGERAK, S. STILGENBAUER, Šárka POSPÍŠILOVÁ, Z. DAVIS, F. FORCONI, F. DAVI, P. GHIA, R. ROSENQUIST and K. STAMATOPOULOS. Immunoglobulin genes in chronic lymphocytic leukemia: key to understanding the disease and improving risk stratification. Haematologica/the hematology journal. Fondazione Ferrata Storti, 2017, vol. 102, No 6, p. 968-971, 5 pp. ISSN 0390-6078. Available from: https://dx.doi.org/10.3324/haematol.2017.165605.

Basic information

• Original name: Immunoglobulin genes in chronic lymphocytic leukemia: key to understanding the disease and improving risk stratification
• Authors: SUTTON, LA. (752 Sweden), A. HADZIDIMITRIOU (300 Greece), P. BALIAKAS (752 Sweden), A. AGATHANGELIDIS (380 Italy), A. LANGERAK (528 Netherlands), S. STILGENBAUER (276 Germany), Šárka POSPÍŠILOVÁ (203 Czech Republic, guarantor, belonging to the institution), Z. DAVIS (826 United Kingdom of Great Britain and Northern Ireland), F. FORCONI (826 United Kingdom of Great Britain and Northern Ireland), F. DAVI (250 France), P. GHIA (380 Italy), R. ROSENQUIST (752 Sweden) and K. STAMATOPOULOS (300 Greece).
• Edition: Haematologica/the hematology journal, Fondazione Ferrata Storti, 2017, 0390-6078.

Other information

• Original language: English
• Type of outcome: Article in a journal
• Field of Study: 30200 3.2 Clinical medicine
• Country of publisher: Italy
• Confidentiality degree: is not subject to a state or trade secret
• Impact factor: Impact factor: 9.090
• RIV identification code: RIV/00216224:14740/17:00095656
• Organization unit: Central European Institute of Technology
• Doi: http://dx.doi.org/10.3324/haematol.2017.165605
• UT WoS: 000402797300009
• Keywords in English: CLL; immunoglobulin; B-cell
• Tags: MEDGENET, rivok
• Tags: International impact, Reviewed

Abstract

While triggering through the B-cell receptor (BcR) facilitates B-cell development and maintenance, it also carries intertwined risks for the emergence of lymphoid malignancies, since malignant B cells can exploit BcR signaling pathways in order to initiate and fuel clonal expansion. Indeed, substantial research into chronic lymphocytic leukemia (CLL), largely based on immunogenetic data, supports the notion that the clonotypic BcR immunoglobulin (IG) engages in the recognition of and selection by putative (auto)antigen.1 This highlights the critical role of the BcR IG in the pathophysiology of CLL and implies that disease development is functionally driven and dynamic, rather than being a simple stochastic process. From a clinical perspective, the remarkable therapeutic efficacy of novel drugs such as ibrutinib and idelalisib which target effectors of the BcR signaling pathway (BTK and PI3K™, respectively), further vouch for this idea, and herald a major paradigm shift which may ultimately lead to changes in the natural history of the disease

Links

• NV15-30015A, research and development project: Name: Analýza klonální heterogenity chronické lymfocytární leukemie pomoci sekvenování nové generace genu pro B-buněčný receptor. Národní studie.
• 692298, interní kód MU: Name: MEDGENET - Medical genomics and epigenomics network (Acronym: MEDGENET)

Investor: European Union, Spreading excellence and widening participation