{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T12:13:37Z","timestamp":1780488817696,"version":"3.54.1"},"reference-count":20,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,11,13]],"date-time":"2024-11-13T00:00:00Z","timestamp":1731456000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,11,13]],"date-time":"2024-11-13T00:00:00Z","timestamp":1731456000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Ratchadapisek Sompoch Fund, Chulalongkorn University","award":["1"],"award-info":[{"award-number":["1"]}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Orphanet J Rare Dis"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of non-Hodgkin lymphoma associated with hemophagocytic lymphohistiocytosis (HLH)\/HLH-like systemic illnesses and germline <jats:italic>HAVCR2<\/jats:italic> mutations. Although previous studies suggested successful treatment of SPTCL with immunosuppressive therapy (IST) without chemotherapy, IST data in pediatric SPTCL remain scarce. To explore characteristics and therapeutic outcomes, comparing IST-based and chemotherapy-based regimens in pediatric SPTCL, in this retrospective cohort study, patients with SPTCL diagnosed at age \u226420 years during 2007-2023 were enrolled from 6 hematology\/oncology centers in Thailand. <jats:italic>HAVCR2<\/jats:italic> exon 2 sequencing was performed using DNA extracted from peripheral blood or bone marrow. Presence of HLH\/HLH-like systemic illnesses, treatment outcomes, and adverse events (AEs) were reviewed and analyzed.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>Of 22 patients with SPTCL (median age at diagnosis, 11.5 years [range, 6.0-19.0]; 63.6% males), 86.4% harbored germline <jats:italic>HAVCR2<\/jats:italic> mutation, either homozygous (77.3%) or heterozygous (9.1%) p.Y82C variant, while 68.2% developed HLH\/HLH-like systemic illnesses. Overall, 36.4% received IST as first-line treatment. Durable complete remission (CR) was achieved in 71.4% and 50.0% after first-line chemotherapy and IST, respectively (<jats:italic>P<\/jats:italic>=0.45); however, chemotherapy tended to increase any AEs compared to IST (57.1% vs. 12.5%; <jats:italic>P<\/jats:italic>=0.07). The most common AEs were hypertension (27.3%), febrile neutropenia (18.2%), and fungal infection (13.6%). Among the relapsed cases, 71.4% could reach CR after subsequent-line therapy. Fatality (4.5%) only occurred in the chemotherapy group.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>Pediatric SPTCL in Thailand frequently involves germline <jats:italic>HAVCR2<\/jats:italic> mutations and\/or HLH\/HLH-like systemic illnesses. With comparable response and modest therapy-related toxicity, IST-based regimens may alternatively be considered as first-line treatment for pediatric SPTCL.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s13023-024-03438-w","type":"journal-article","created":{"date-parts":[[2024,11,13]],"date-time":"2024-11-13T06:01:36Z","timestamp":1731477696000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Characteristics and therapeutic outcomes of subcutaneous panniculitis-like T-cell lymphoma with and without germline HAVCR2 mutations in Thai children and adolescents"],"prefix":"10.1186","volume":"19","author":[{"given":"Pimpitcha","family":"Youthong","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8118-163X","authenticated-orcid":false,"given":"Samart","family":"Pakakasama","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6804-9121","authenticated-orcid":false,"given":"Patcharee","family":"Komvilaisak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5006-9810","authenticated-orcid":false,"given":"Piya","family":"Rujkijyanont","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2520-1162","authenticated-orcid":false,"given":"Chane","family":"Choed-Amphai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2101-2206","authenticated-orcid":false,"given":"Kamon","family":"Phuakpet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6257-0867","authenticated-orcid":false,"given":"Chatphatai","family":"Moonla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6949-4449","authenticated-orcid":false,"given":"Chantana","family":"Polprasert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1097-3482","authenticated-orcid":false,"given":"Darintr","family":"Sosothikul","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,11,13]]},"reference":[{"issue":"20","key":"3438_CR1","doi-asserted-by":"publisher","first-page":"2375","DOI":"10.1182\/blood-2016-01-643569","volume":"127","author":"SH Swerdlow","year":"2016","unstructured":"Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016;127(20):2375\u201390.","journal-title":"Blood."},{"issue":"12","key":"3438_CR2","doi-asserted-by":"publisher","first-page":"3261","DOI":"10.3324\/haematol.2023.282718","volume":"108","author":"NE Lewis","year":"2023","unstructured":"Lewis NE, Zhou T, Dogan A. Biology and genetics of extranodal mature T-cell and NK-cell lymphomas and lymphoproliferative disorders. Haematologica. 2023;108(12):3261\u201377.","journal-title":"Haematologica."},{"key":"3438_CR3","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1097\/00000478-199101000-00002","volume":"15","author":"CL Gonzalez","year":"1991","unstructured":"Gonzalez CL, Medeiros LJ, Braziel RM, Jaffe ES. T-cell lymphoma involving subcutaneous tissue. A clinicopathologic entity commonly associated with hemophagocytic syndrome. 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Written informed consent forms were obtained from each patient and\/or their parents\/guardians prior to participation in the study, in accordance with the Declaration of Helsinki.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval and consent to participate"}},{"value":"Not applicable","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"426"}}