TY - JOUR
T1 - Chronic lymphocytic leukaemia
AU - Kipps, Thomas J.
AU - Stevenson, Freda K.
AU - Wu, Catherine J.
AU - Croce, Carlo M.
AU - Packham, Graham
AU - Wierda, William G.
AU - O'Brien, Susan
AU - Gribben, John
AU - Rai, Kanti
N1 - Funding Information:
University of California San Diego, for help in figure development. This Primer is supported by the US NIH PO1-CA81534 Leukemia & Lymphoma Society (LLS) Specialized Center of Research Program (SCOR) grant (T.J.K.), 1R01HL116452, 1R01CA182461, CLL Global Reseach Foundation (W.G.W.), the Blood Cancer Research Fund (T.J.K.), and U10CA180861.
Publisher Copyright:
© 2017 Macmillan Publishers Limited, part of Springer Nature. All rights reserved.
PY - 2017/1/19
Y1 - 2017/1/19
N2 - Chronic lymphocytic leukaemia (CLL) is a malignancy of CD5 + B cells that is characterized by the accumulation of small, mature-Appearing lymphocytes in the blood, marrow and lymphoid tissues. Signalling via surface immunoglobulin, which constitutes the major part of the B cell receptor, and several genetic alterations play a part in CLL pathogenesis, in addition to interactions between CLL cells and other cell types, such as stromal cells, T cells and nurse-like cells in the lymph nodes. The clinical progression of CLL is heterogeneous and ranges from patients who require treatment soon after diagnosis to others who do not require therapy for many years, if at all. Several factors, including the immunoglobulin heavy-chain variable region gene (IGHV) mutational status, genomic changes, patient age and the presence of comorbidities, should be considered when defining the optimal management strategies, which include chemotherapy, chemoimmunotherapy and/or drugs targeting B cell receptor signalling or inhibitors of apoptosis, such as BCL-2. Research on the biology of CLL has profoundly enhanced our ability to identify patients who are at higher risk for disease progression and our capacity to treat patients with drugs that selectively target distinctive phenotypic or physiological features of CLL. How these and other advances have shaped our current understanding and treatment of patients with CLL is the subject of this Primer.
AB - Chronic lymphocytic leukaemia (CLL) is a malignancy of CD5 + B cells that is characterized by the accumulation of small, mature-Appearing lymphocytes in the blood, marrow and lymphoid tissues. Signalling via surface immunoglobulin, which constitutes the major part of the B cell receptor, and several genetic alterations play a part in CLL pathogenesis, in addition to interactions between CLL cells and other cell types, such as stromal cells, T cells and nurse-like cells in the lymph nodes. The clinical progression of CLL is heterogeneous and ranges from patients who require treatment soon after diagnosis to others who do not require therapy for many years, if at all. Several factors, including the immunoglobulin heavy-chain variable region gene (IGHV) mutational status, genomic changes, patient age and the presence of comorbidities, should be considered when defining the optimal management strategies, which include chemotherapy, chemoimmunotherapy and/or drugs targeting B cell receptor signalling or inhibitors of apoptosis, such as BCL-2. Research on the biology of CLL has profoundly enhanced our ability to identify patients who are at higher risk for disease progression and our capacity to treat patients with drugs that selectively target distinctive phenotypic or physiological features of CLL. How these and other advances have shaped our current understanding and treatment of patients with CLL is the subject of this Primer.
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U2 - 10.1038/nrdp.2016.96
DO - 10.1038/nrdp.2016.96
M3 - Article
C2 - 28102226
AN - SCOPUS:85009999724
SN - 2056-676X
VL - 3
JO - Nature Reviews Disease Primers
JF - Nature Reviews Disease Primers
M1 - 16096
ER -