SOHO State of the Art Updates and Next Questions | Challenging Cases in Rare T-Cell Lymphomas |
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| Affiliation: | 1. H. Lee Moffitt Cancer Center and Research Institute, Department of Malignant Hematology, Tampa, FL;2. Memorial Sloan Kettering Cancer Center, Division of Multiple Myeloma and Lymphoma, Montvale, NJ;3. Flatiron Health, Division of Research Oncology and Real World Evidence, New York, NY;4. Translational Genomics Research Institute, City of Hope Cancer Center, Phoenix, AZ;5. International Myeloma Foundation, Chief Medical Officer, Studio City, CA;1. Department of Hematology, Jagiellonian University Medical College, Kraków, Poland;2. Department of Hematology, Blood Neoplasms and Bone Marrow Transplantation, Wroclaw Medical University, Wroclaw, Poland;3. Hackensack University Medical Center, New Jersey Medical School, Rutgers University, Hackensack, NJ;1. Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, New York, NY;2. Atrium Health Wake Forest Baptist Comprehensive Cancer Center; Winston-Salem, NC;1. Pediatric Blood Diseases Center, State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Haihe Laboratory of Cell Ecosystem, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin, China;2. Tianjin Institutes of Health Science, Tianjin, China;3. Department of Hematology and Oncology, Children''s Hospital of Nanjing Medical University, Nanjing, China;4. Division of Hematology/Oncology, Children''s Hospital of Zhejiang University School of Medicine, Hangzhou, China;5. Departments of Oncology, Global Pediatric Medicine and Pathology, St. Jude Children''s Research Hospital, Memphis, TN;1. Department of Hematology, Université de Lille, CHU Lille, Lille, France;2. Department of Clinical Therapeutics, School of Medicine, National and Kapodistrian University of Athens, Alexandra General Hospital, Athens, Greece;3. Department of Hematology, Hôpital Haut Lévêque, University Hospital, Pessac, France;4. Department of Hematology and Cellular Therapy, CIC U1402 CHU de Poitiers, Poitiers, France;5. Department of Hematology, UZ Leuven, Leuven, Belgium;6. Department of Oncology, Hematology and Bone Marrow Transplantation with Department of Pneumonology, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany;7. Worldwide Medical Affairs, Celgene International Sàrl, a Bristol-Myers Squibb Company, Boudry, Switzerland;8. Worldwide Medical Affairs, Celgene, a Bristol-Myers Squibb Company, Princeton, NJ |
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| Abstract: | Mature T- and NK-cell neoplasms (MTNKN) collectively represent a rare disorder, representing less than 15% of all non-Hodgkin lymphoma (NHL) cases and qualifying for orphan disease designation by the U.S. Food and Drug Administration (FDA). These consist of 9 families in the fifth revised WHO classification of lymphoid neoplasms, which are made up of over 30 disease subtypes, underscoring the heterogeneity of clinical features, molecular biology, and genetics across this disease group. Moreover, the 5 most common subtypes (peripheral T-cell lymphoma, not otherwise specified; nodal TFH cell lymphoma, angioimmunoblastic type; extranodal NK-cell/T-cell lymphoma; adult T-cell leukemia/lymphoma; and ALK-positive or -negative anaplastic large cell lymphoma) comprise over 75% of MTNKN cases, so other subtypes are exceedingly rare in the context of all NHL diagnoses and consequently often lack consensus on best practices in diagnosis and management. In this review, we discuss the following entities–enteropathy-associated T-cell lymphoma (EATL), monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), hepatosplenic T-cell lymphoma (HSTCL), subcutaneous panniculitis-like T-cell lymphoma (SPTCL), and primary cutaneous ɣδ T-cell lymphoma (PCGD-TCL) – with an emphasis on clinical and diagnostic features and options for management. |
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