![]() |
![]() |
![]() |
|
Chapter 19
Chronic lymphocytic leukemia
Chronic lymphocytic leukemia
Incidence, epidemiology, and demographics
B-cell chronic lymphocytic leukemia (CLL) is a lymphoproliferative disorder manifested by a clonal expansion of mature, long-lived, B lymphocytes. The disease is identical to small lymphocytic leukemia (SLL); the clinical distinction is whether the leukemic (blood and marrow) or the nodal components of the disease predominate in a given patient. The 2001 World Health Organization classification scheme considers CLL and SLL in one category (CLL/SLL) because of shared clinicopathologic features. The mechanisms of why clonal B cells predominate in marrow or node are not clearly understood, but one possibility may be the ability of B lymphocytes to "home" to certain Familial CLL
Diagnosis
Differential diagnosis: cytogenetic, immunophenotypic, and molecular aspects Is there a minimum level of blood B cells necessary for diagnosis of CLL? Monoclonal B-cell lymphocytosis
Clinical and laboratory features
Staging
Prognostic factors
The case for prognostic factors in CLL Traditional prognostic factors Novel biomarker prognostic factors in assessing high-risk subgroups of patients with CLL IgVH mutation CD38, CD49d, and ZAP-70 Fluorescence in situ hybridization Practical use of prognostic markers (traditional or novel) for newly diagnosed CLL versus previously treated or relapsed/refractory CLL Prognostic models Multivariate model using traditional prognostic factors Multivariate models using novel biomarkers
Therapy
Initial therapy Alkylator-based therapy Purine analogs Rituximab Combination purine analog–based chemotherapy and chemoimmunotherapy Other purine analogs Alemtuzumab Bendamustine Relapsed/refractory CLL: workup and treatment options
Approach to newly diagnosed patients with high-risk disease
Options for the relapsed/refractory CLL patient Lenalidomide Flavopiridol HuMax-CD20 (ofatumumab) Stem cell transplantation
Complications of CLL
Autoimmune complications Infectious complications Transformation to prolymphocytic leukemia or Richter transformation Other second malignancies
Quality-of-life issues
Other indolent cell leukemias
Prolymphocytic leukemia Hairy cell leukemia Marginal zone lymphoma Lymphoplasmacytic lymphoma T-cell CLL and other chronic T-cell leukemias Mantle cell lymphoma
The future
| ||||||||||||||||||||||||||||||||||||||||||||||||||