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INTRODUCTION

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  • The term peripheral T-cell lymphoma (PTCL) refers to lymphomas originating in a mature (ie, post-thymic) T-cell.

  • PTCLs make up 10% to 15% of all non-Hodgkin lymphomas and represent at least 23 heterogeneous diseases.

  • Table 66–1 lists the most common of the mature T-cell and natural killer (NK) cell lymphomas.

  • The incidence of various types of PTCL varies widely based on geography (Table 66–2).

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Table Graphic Jump Location
TABLE 66–12008 WHO CLASSIFICATION OF MATURE T-CELL AND NATURAL KILLER-CELL NEOPLASMS (EXCLUDING PRIMARY CUTANEOUS LYMPHOMA)
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Table Graphic Jump Location
TABLE 66–2INCIDENCE OF LYMPHOMA SUBTYPES BY GEOGRAPHIC REGION
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PERIPHERAL T-CELL LYMPHOMA

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  • The diagnosis of PTCL is based on histologic features, immunophenotype, molecular studies, and clinical presentation.

  • B-cell lymphomas are characterized by immunophenotypic features, whereas T-cell lymphomas are characterized by antigen aberrancy, which may vary within a subtype and over the course of the disease.

  • Pathologists have a low concordance rate when reviewing the histopathology of a PTCL as compared to a mature B-cell lymphoma.

  • In the diagnosis of PTCL it is important to exclude a reactive process, particularly when the clinical picture is not congruent with the pathological features, ...

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