2018JSH实践指南：血液恶性肿瘤——外周T细胞淋巴瘤.pdf

International Journal of Hematology /10.1007/s12185-018-02589-4 GUIDE LINE JSH practical guidelines for hematological malignancies, 2018: 7. Peripheral T-cell lymphoma (PTCL) 1 2 Motoko Yamaguchi  · Ritsuro Suzuki Received: 28 November 2018 / Accepted: 28 November 2018 © Japanese Society of Hematology 2019 Overview LDH level > institutional upper limit of normal, and bone marrow involvement [5]. The 2017 WHO Classifcation lists approximately 30 disease The Lugano classifcation (2014) also includes response entities under the category of peripheral T-cell lymphomas criteria for PTCL that incorporates positron-emission (PTCLs) and natural killer (NK) cell neoplasms [1]. The tomography fndings [4]. entity-specifc prevalence is known to difer between world Note: rare PTCL entities accounting for less than 5% of regions. The International T-Cell Lymphoma Project, a all T/NK-cell neoplasms in the International T-Cell Lym- global retrospective study of approximately 1300 patients, phoma Project include enteropathy-associated T-cell lym- found that the most prevalent PTCL entities in western phoma (EATL), hepatosplenic T-cell lymphoma (HSTL), countries, from highest to lowest prevalence, were PTCL primary cutaneous ALCL, and subcutaneous panniculitis- not otherwise specifed (PTCL-NOS), angioimmunoblastic like T-cell lymphoma [2]. EATL accounts for 9.1% of all T-cell lymphoma (AITL), ALK-positive anaplastic large lymphomas in Europe, where celiac disease is prevalent, and cell lymphoma (ALCL), and ALK-negative ALCL [2]. This for 5.8% in North America, but is uncommon in Asia at only chapt