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Angioimmunoblastic T‐cell Lymphoma

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AITL is thought to arise from follicular helper T cells which correspond to a subset of peripheral CD4 positive T cells [22, 23]. It is the second most commonly seen subtype of PTCL. The highest incidence rates are in Europe, specially Spain, France, Norway, Germany, Italy, and the UK (28.7% of PTCLs), while lower rates are seen in North America (16% of PTCLs) and Asia (17.9% of PTCLs) [10, 24]. In the United States, the incidence is approximately 0.05 cases per 100 000 person years [25] and it is more frequently seen in Asian/Pacific Islanders and Hispanic Whites, compared with non‐Hispanic Whites and African Americans [18]. AITL is not commonly seen in American Indian/Alaskan natives.

Typically, the disease affects older adults with a median age at diagnosis of 65 years in France [26] and 69 years in the United States [27]. It is almost never seen in persons less than 20 years of age, and there is a slight male preponderance seen is some studies [28–31]. Although Epstein–Barr virus (EBV) infection is associated with pathogenesis of AITL, the exact mechanism remains a topic of debate [32]. Electrical fitters and persons with a family history of hematological malignancies appear to carry an increased risk of developing AITL [33].

The Peripheral T-Cell Lymphomas

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