Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ INTRODUCTION ++ 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 28 heterogeneous diseases as currently classified. Table 67–1 lists the mature T-cell and natural killer (NK) cell lymphomas excluding primary cutaneous lymphoma. The four most common entities make up 60% to 65% of cases in the United States and northern Europe; these are PTCL not otherwise specified (PTCL-NOS), angioimmunoblastic T-cell lymphoma (AITL), anaplastic lymphoma kinase positive (ALK+) anaplastic large-cell lymphoma (ALCL), and ALK-negative ALCL. The incidence of various types of PTCL varies widely based on geography (Table 67–2). ++Table Graphic Jump LocationTABLE 67–12016 WORLD HEALTH ORGANIZATION CLASSIFICATION OF MATURE T-CELL AND NATURAL KILLER–CELL NEOPLASMS (EXCLUDING PRIMARY CUTANEOUS LYMPHOMAS)View Table||Download (.pdf) TABLE 67–1 2016 WORLD HEALTH ORGANIZATION CLASSIFICATION OF MATURE T-CELL AND NATURAL KILLER–CELL NEOPLASMS (EXCLUDING PRIMARY CUTANEOUS LYMPHOMAS) Peripheral T-cell lymphoma, NOS Angioimmunoblastic T-cell lymphoma Follicular T-cell lymphomaa Anaplastic large-cell lymphoma, ALK-positive Anaplastic large-cell lymphoma, ALK-negative Breast implant–associated anaplastic large-cell lymphomaa Enteropathy-associated T-cell lymphoma Monomorphic epitheliotropic intestinal T-cell lymphoma Adult T-cell leukemia/lymphoma Hydroa vacciniforme–like lymphoma T-cell prolymphocytic leukemia T-cell large granular lymphocytic leukemia Hepatosplenic T-cell lymphoma Extranodal NK-/T-cell lymphoma, nasal type Aggressive NK-cell leukemia Systemic EBV+ T-cell lymphoproliferative disease of childhood (associated with chronic active EBV infection) Chronic lymphoproliferative disorder of NK cellsa ALK, anaplastic lymphoma kinase; EBV, Epstein-Barr virus; NK, natural killer; NOS, not otherwise specified.aProvisional entity. ++Table Graphic Jump LocationTABLE 67–2INCIDENCE OF LYMPHOMA SUBTYPES BY GEOGRAPHIC REGIONView Table||Download (.pdf) TABLE 67–2 INCIDENCE OF LYMPHOMA SUBTYPES BY GEOGRAPHIC REGION Subtype Registry PTCL-NOS AITL ALCL ALK+ ALCL ALK– NK/T ATL EATL North America IPTCL 34% 16% 16% 8% 5% 2% 6% BCCA 59% 5% 6% 9% 9% NAa 5% COMPLETE 34% 15% 11% 8% 6% 2% 3% Europe IPTCL 34% 29% 6% 9% 4% 1% 9% Swedish 34% 14% 9% 15% 4% NAa 9% Asia IPTCL 22% 18% 3% 3% 22% 25% 2% AITL, angioimmunoblastic T-cell lymphoma; ALCL ALK–, anaplastic large-cell lymphoma anaplastic lymphoma kinase negative; ALCL ALK+, anaplastic large-cell lymphoma anaplastic lymphoma kinase positive; ATL, adult T-cell leukemia/lymphoma; BCCA, British Columbia Cancer Agency; COMPLETE, Comprehensive Oncology Measures for Peripheral T-Cell Lymphoma Treatment; EATL, enteropathy-associated T-cell lymphoma; IPTCL, International Peripheral T-Cell Lymphoma Project; NA, not available; NK/T, natural killer–cell/T-cell lymphoma; NOS, not otherwise specified; PTCL, peripheral T-cell lymphoma.aATL patients were excluded in both the BCCA and Swedish Registry Studies. +++ GENERAL ASPECTS OF PERIPHERAL T-CELL LYMPHOMAS ++ 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 ... Your Access profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Password? Forgot Username? Sign in via OpenAthens Sign in via Shibboleth