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Mature T-cell lymphomas comprise a biologically heterogeneous group of diseases defined by their histopathologic and clinical characteristics. They account for approximately 10 to 15 percent of all lymphoid malignancies. There is considerable variation in incidence worldwide with respect to race and region. Although the reason for this is not known, in some instances specific viral infections are linked to selected subtypes. In general, these lymphomas are biologically more aggressive and less responsive to conventional chemotherapy than their B-cell counterparts, but have a highly variable course. With the exception of anaplastic large cell lymphoma, the role of intensive chemotherapy remains largely undefined,1 prompting many to examine the potential utility of targeted therapies in these diseases.

Acronyms and Abbreviations

Abbreviations and acronyms used in this chapter include: ADCC, antibody-dependent cell-mediated cytotoxicity; AILD, angioimmunoblastic lymphadenopathy with dysproteinemia; AITL, angioimmunoblastic T-cell lymphoma; ALCL, anaplastic large cell lymphoma; CHOP, cyclophosphamide, hydroxydaunorubicin (doxorubicin), vincristine (Oncovin), prednisone; CR, complete remission; EBV, Epstein-Barr virus; HTLV, human T-lymphotropic virus; IPI, international prognostic index; LGL, larger granular lymphocyte; MACOP-B, high-dose methotrexate, doxorubicin, cyclophosphamide, vincristine, prednisone, bleomycin; mAb, monoclonal antibody; NK, natural killer; PCR, polymerase chain reaction; PR, partial remission; PTCL, peripheral T-cell lymphoma; RANKL, receptor activator of nuclear factor-κB ligand; RANTES, regulated upon activation, normal T-cell expressed and secreted; REAL, revised European-American classification of lymphoid neoplasm; TARC, thymus and activation-regulated chemokine; TCR, T-cell receptor; VEPA, vincristine, cyclophosphamide, prednisolone, and doxorubicin; VEPA-M, VEPA plus methotrexate; WHO, World Health Organization.

The classification of mature T-cell and natural killer (NK)-cell lymphomas has undergone reorganization over the last 15 years. Initially, the working formulation, based on morphology, did not define lymphomas based on cell of origin.2 In 1994, the revised European-American classification of lymphoid neoplasm (REAL) was developed by the International Lymphoma Study Group, and although largely descriptive, incorporated cell lineages and clinical features and was the first classification to designate T-cell lymphomas.3 The REAL classification further divided T-cell lymphomas into two groups based on the extent of maturation, the precursor T-cell lymphomas, and peripheral T-cell lymphomas. T-cell lymphomas were further subdivided by clinical behavior and risk (low, intermediate, and high risk).

In 2001, the European Association for Haematopathology and the Society for Hematopathology jointly revised the REAL classification, creating the World Health Organization (WHO) classification of Tumors of the Haematopoietic and Lymphoid in use today and further described in Chaps. 92 and 98. There are 14 major disease categories of mature T-cell and NK cell neoplasms, defined on the basis of anatomical disease distribution and the putative cell of origin (Table 106–1).4 Chap. 105 discusses the cutaneous T-cell lymphomas, mycosis fungoides, Sézary syndrome, and primary cutaneous CD30-positive T-cell lymphoproliferative disorders.

Table 106–1. WHO Classification of Mature T- and NK-Cell Neoplasms

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