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Chronic lymphocytic leukaemia 

Chronic lymphocytic leukaemia

Chapter:
Chronic lymphocytic leukaemia
Author(s):

Clive S. Zent

and Aaron Polliack

DOI:
10.1093/med/9780199204854.003.220305_update_002

Update:

Reviewed June 2013—no significant changes required.

Updated on 29 Aug 2013. The previous version of this content can be found here.
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date: 30 March 2017

Chronic lymphocytic leukaemia/small lymphocytic lymphoma (CLL) is the most prevalent lymphoid neoplasm in Europe and North America. The ‘cell of origin’ is a mature B lymphocyte that has a rearranged immunoglobulin gene. CLL cells express modest amounts of surface immunoglobulin, and are characterized by defective apoptosis. The cause of CLL is unknown.

Clinical features

Most patients show no specific clinical features of disease and diagnosis is made incidentally after finding peripheral blood lymphocytosis. A proportion of patients have rapidly progressive disease and develop symptoms resulting from (1) tissue accumulation of lymphocytes such as disfiguring lymphadenopathy, splenomegaly with abdominal discomfort, profound fatigue, drenching night sweats, weight loss, and fever; or (2) manifestations of marrow failure with cytopenias including anaemia, and thrombocytopenia. Patients also exhibit an increased risk of infection and can develop autoimmune cytopenias and second malignancies, including transformation to more aggressive types of lymphoid neoplasia, like diffuse large B cell lymphoma (Richter’s syndrome).

Diagnosis and clinical staging

Diagnosis is usually made by analysis of the immunophenotype of the circulating leukaemic cells from the blood, or in rare cases without a detectable circulating monoclonal B cell population, from the bone marrow, lymph nodes or spleen. Current diagnostic criteria have an arbitrary requirement for (1) a monoclonal B-lymphocyte count >5 × 109/litre, or (2) clinically detectable adenopathy—of at least 1 cm in diameter, or (3) organomegaly, or (4) over 30% bone marrow involvement by CLL cells. Staging is based on both clinical examination and blood count evaluation.

Treatment and prognosis

Treatment—there is no standard curative therapy, and patients should not be treated until they have progressive and symptomatic disease or develop anaemia or thrombocytopenia due to bone marrow failure. If a decision is made to treat, then the best initial treatment is chemo-immunotherapy combining purine analogues, alkylating agents, and monoclonal antibodies in patients that are sufficiently fit to undergo this therapy and do not have p53 pathway dysfunction.

Treatment approaches for frail patients and patients with progressive CLL associated with 17p deletion and p53 pathway dysfunction remain an open issue and no established guidelines have been defined as yet.

Prognosis—this is highly variable and depends on the clinical stage of disease, biological characteristics of the CLL cells, and the general health and performance status of the patient. The median survival at diagnosis is in excess of 10 years, but is considerably shorter (about 6 years) in patients with advanced stage disease at diagnosis.

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