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Chapter 12
Lymphoproliferative disorders
Overview of lymphocyte development
Lymphocytes develop from a common lymphoid progenitor cell. B cells mature primarily in the bone marrow, whereas T cells mature in the thymus. Although the process and signals of maturation differ between the 2 cell types, they rely on similar genetic events to generate specific antibodies or cell surface receptors. These gene rearrangements are critical for the development of a broad immune repertoire and also provide molecular markers of clonality that can be used to diagnose lymphoid malignancies.
B-cell development
B-cell maturation consists of early (antigen-independent) and late (antigen-dependent) stages. Early development is initiated by the rearrangement of genes for the heavy T-cell development Biology of lymphomatous transformation Diagnostic testing of lymphoproliferative disorders Morphology Immunophenotyping Molecular genetics/cytogenetis Classification of lymphocytic malignancies
Chronic lymphocytic leukemia
Molecular, cytogenetic, and immunophenotypic studies Clinical and laboratory features Diagnostic criteria and differential diagnosis Staging and prognosis Immunologic abnormalities in CLL Treatment indications and response criteria CLL Response criteria Cytotoxic therapy Monoclonal antibody therapy Stem cell transplantation Selection of appropriate therapy Prognosis and transformation Prolymphocytic leukemia T-cell CLL Lymphoproliferative disorder of granular lymphocytes Hairy cell leukemia
Non-Hodgkin lymphoma
Pathology Epidemiology, pathogenesis, and molecular characterization Staging and prognostic factors Indolent B-cell NHL Follicular lymphoma and the spectrum of indolent NHL Marginal zone lymphomas MALT lymphomas Nodal marginal zone B-cell lymphoma Lymphoplasmacytic lymphoma (Waldenström macroglobulinemia) Peripheral T-cell lymphomas Aggressive NHL Diffuse large B-cell lymphoma Dose dense therapy in DLBCL Localized aggressive lymphoma Assessment of therapeutic response and patient follow-up Relapsed aggressive lymphoma Mediastinal (thymic) large B-cell lymphoma Burkitt lymphoma Mantle cell lymphoma T-cell large cell lymphoma Hepatosplenic / T-cell lymphomaAnaplastic large cell lymphoma
Immunodeficiency-associated lymphoproliferative disorders
Primary central nervous system lymphoma Non-Hodgkin lymphomas in children
Hodgkin lymphoma
Pathology/staging Treatment
Early stage
Advanced disease Relapsed/refractory disease Hematopoietic stem cell transplant Management of lymphocyte-predominant Hodgkin disease Hodgkin lymphoma in children Long-term complications of Hodgkin lymphoma therapy | |||||||||||||||||||||||||||||||||||||||||||||||||||