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Nat Rev Dis Primers. 2017 Jan 19;3:16096. doi: 10.1038/nrdp.2016.96.

Chronic lymphocytic leukaemia.

Author information

1
Division of Hematology-Oncology, Department of Medicine, Moores Cancer Centre, University of California, San Diego, 3855 Health Sciences Drive M/C 0820, La Jolla, California 92093, USA.
2
Southampton Cancer Research UK Centre, Cancer Sciences Academic Unit, Faculty of Medicine, University of Southampton, Southampton, UK.
3
Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
4
Department of Molecular Virology, Immunology and Medical Genetics, Ohio State University, Columbus, Ohio, USA.
5
Department of Hematology, MD Anderson Cancer Centre, Houston, Texas, USA.
6
Division of Hematology, Department of Medicine, University of California, Irvine, California, USA.
7
Department of Haemato-Oncology, Barts Cancer Institute, Queen Mary University of London, London, UK.
8
CLL Research and Treatment Program, Feinstein Institute for Medical Research, Northwell Health, New Hyde Park, New York, USA.

Abstract

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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