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Chapter 67: Mature T-Cell and Natural Killer Cell Lymphomas

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 67–12016 WORLD HEALTH ORGANIZATION CLASSIFICATION OF MATURE T-CELL AND NATURAL KILLER–CELL NEOPLASMS (EXCLUDING PRIMARY CUTANEOUS LYMPHOMAS)
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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 67–2INCIDENCE OF LYMPHOMA SUBTYPES BY GEOGRAPHIC REGION
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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 ...

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