JSH practical guidelines for hematological malignancies, 2018: 7. Peripheral T-cell lymphoma (PTCL)
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The 2017 WHO Classification lists approximately 30 disease entities under the category of peripheral T-cell lymphomas (PTCLs) and natural killer (NK) cell neoplasms . The entity-specific prevalence is known to differ between world regions. The International T-Cell Lymphoma Project, a global retrospective study of approximately 1300 patients, found that the most prevalent PTCL entities in western countries, from highest to lowest prevalence, were PTCL not otherwise specified (PTCL-NOS), angioimmunoblastic T-cell lymphoma (AITL), ALK-positive anaplastic large cell lymphoma (ALCL), and ALK-negative ALCL . This chapter covers these four entities. Two other PTCL entities common in Japan, adult T-cell leukemia–lymphoma (ATL) and extranodal NK/T-cell lymphoma, nasal type (ENKL), will be covered in another chapter. Cutaneous T-cell lymphoma (CTCL) is covered in a separate set of guidelines (Evidence-based Guidelines for the Treatment of Malignant Skin Tumors, 2nd Edition) .