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).
TABLE 66–12008 WHO CLASSIFICATION OF MATURE T-CELL AND NATURAL KILLER-CELL NEOPLASMS (EXCLUDING PRIMARY CUTANEOUS LYMPHOMA) |Favorite Table|Download (.pdf) TABLE 66–1 2008 WHO CLASSIFICATION OF MATURE T-CELL AND NATURAL KILLER-CELL NEOPLASMS (EXCLUDING PRIMARY CUTANEOUS LYMPHOMA)
Peripheral T-cell lymphoma, NOS
Angioimmunoblastic T-cell lymphoma
Anaplastic large cell lymphoma, ALK-positive
Anaplastic large cell lymphoma, ALK-negative
Enteropathy-associated 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 cells*
TABLE 66–2INCIDENCE OF LYMPHOMA SUBTYPES BY GEOGRAPHIC REGION |Favorite Table|Download (.pdf) TABLE 66–2 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% ||NA* ||5% |
| ||COMPLETE ||34% ||15% ||11% ||8% ||6% ||2% ||3% |
|Europe ||IPTCL ||34% ||29% ||6% ||9% ||4% ||1% ||9% |
| ||Swedish ||34% ||14% ||9% ||15% ||4% ||NA* ||9% |
|Asia ||IPTCL ||22% ||18% ||3% ||3% ||22% ||25% ||2% |
PERIPHERAL T-CELL LYMPHOMA
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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