Publication details
Immunoglobulin genes in chronic lymphocytic leukemia: key to understanding the disease and improving risk stratification
| Authors | |
|---|---|
| Year of publication | 2017 |
| Type | Article in Periodical |
| Magazine / Source | Haematologica/the hematology journal |
| MU Faculty or unit | |
| Citation | |
| Doi | http://dx.doi.org/10.3324/haematol.2017.165605 |
| Field | Oncology and hematology |
| Keywords | CLL; immunoglobulin; B-cell |
| Description | 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 |
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