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Peripheral T‐cell Lymphoma, Not Otherwise Specified

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PTCL‐NOS is a heterogeneous group of predominantly nodal T‐cell lymphomas that originate from different types of mature T cells and do not meet the criteria for other specially defined subtypes of PTCL. This is the most prevalent subtype of PTCL seen in Western countries, accounting for approximately 30% of PTCL and approximately 4% of NHLs overall [8, 9, 14–16]. Owing to improved diagnostic methods and awareness of the disease, the incidence of PTCL‐NOS has increased in the United States from approximately 0.1 cases per 100 000 population in 1992 to approximately 0.4 cases per 100 000 population in recent years [17]. In the United States, African Americans have a higher incidence, compared with Asian/Pacific Islanders, Hispanic Whites, and Non‐Hispanic Whites, with American Indian/Alaskan natives having the lowest incidence [18]. As PTCLs account for approximately 15–20% of all NHLs in Asia, the incidence of PTCL‐NOS is higher in Asia compared with the Western world [10].

Typically, the median age at diagnosis is 60–65 years [10, 18], and men have a higher incidence than women, with a ratio of approximately two to one [19].

A history of alcohol consumption and allergies is associated with decreased risk, whereas cigarette smoking, a history of psoriasis, and celiac disease are associated with higher risk for the development of PTCL‐NOS per the InterLymph study [20]. In African Americans, an inverse relationship between PTCL‐NOS and sun exposure was reported in one study [21].

Table 5.1 Major lymphoma subtypes by geographic region

Source: Vose et al. [10].

Subtype Incidence (%)
North America Europe Asia
PTCL‐NOS 34.4 34.3 22.4
Angioimmunoblastic 16.0 28.7 17.9
ALK+ ALCL 16.0 6.4 3.2
ALK– ALCL 7.8 9.4 2.6
NKTCL 5.1 4.3 22.4
ATLL 2.0 1.0 25.0
Enteropathy‐type 5.8 9.1 1.9
Hepatosplenic 3.0 2.3 0.2
Primary cutaneous ALCL 5.4 0.8 0.7
Subcutaneous panniculitis‐like 1.3 0.5 1.3
Unclassifiable T‐cell 2.3 3.3 2.4

ALCL, anaplastic large‐cell lymphoma; ALK, anaplastic lymphoma kinase; ATLL, adult T‐cell leukemia/lymphoma; NKTCL, natural killer/T‐cell lymphoma; NOS, not otherwise specified; PTCL, peripheral T‐cell lymphoma.

The Peripheral T-Cell Lymphomas

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