24. Chronic Lymphocytic Leukemia: Pathophysiology, Diagnosis and Manifestations, and Prognostic Markers

  1. Stefan Faderl MD Associate Professor and
  2. Hagop Kantarjian MD Chairman Professor
  1. Thorsten Zenz MD,
  2. Hartmut Döhner MD and
  3. Stephan Stilgenbauer MD

Published Online: 4 JAN 2011

DOI: 10.1002/9781444327359.ch24

Leukemias: Principles and Practice of Therapy

Leukemias: Principles and Practice of Therapy

How to Cite

Zenz, T., Döhner, H. and Stilgenbauer, S. (2010) Chronic Lymphocytic Leukemia: Pathophysiology, Diagnosis and Manifestations, and Prognostic Markers, in Leukemias: Principles and Practice of Therapy (eds S. Faderl and H. Kantarjian), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444327359.ch24

Editor Information

  1. Department of Leukemia, University of Texas, MD Anderson Cancer Center, Houston, TX, USA

Author Information

  1. Department of Internal Medicine III, University of Ulm, Ulm, Germany

Publication History

  1. Published Online: 4 JAN 2011
  2. Published Print: 26 NOV 2010

ISBN Information

Print ISBN: 9781405182355

Online ISBN: 9781444327359

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Keywords:

  • chronic lymphocytic leukemia (CLL);
  • diagnosis;
  • immunophenotyping;
  • staging;
  • prognosis;
  • cytogenetics;
  • immunoglobulin heavy chain variable (IGHV) mutational status;
  • ZAP70;
  • 17p deletion

Summary

The monoclonal population of B cells in chronic lymphocytic leukemia (CLL) express CD19, CD5, and CD23 and have reduced levels of membrane immunoglobulins (Igs), a phenotype of mature, activated B lymphocytes. The malignant B cells usually accumulate over years and lead to clinically diverse manifestations. Some patients with CLL survive for many years without therapy, whereas others have a rapidly fatal disease, despite aggressive therapy. This heterogeneity is partly accounted for by the staging systems of Rai and Binet, which classify patients according to tumor burden and hematopoietic impairment. Within the past decade, CLL has been shown to be a biologically diverse disorder. Its heterogeneity reflects differences in genetic the make-up of the tumor cell with genomic aberrations and mutation status of immunoglobulin heavy chain variable (IGHV) genes (or surrogate markers thereof). These genetic markers separate prognostic subgroups, but also help to decipher the biology and pathophysiology of the disease.