皮下脂膜炎样T细胞淋巴瘤研究进展

doi:10.35541/cjd.20230172

Abstract

Abstract: 【Abstract】 High-frequency mutations in the hepatitis A virus cellular receptor 2 gene have been detected in subcutaneous panniculitis-like T-cell lymphoma, which paves a new way for studying the pathogenesis of this disorder, and provides a basis for its diagnosis, treatment, and prognosis assessment. This review summarizes the latest research achievements in subcutaneous panniculitis-like T-cell lymphoma.

Key words: Leukemia-lymphoma, adult T-cell, Mutation, Diagnosis, differential, Drug therapy, Prognosis, Subcutaneous panniculitis-like T-cell lymphoma, Panniculitis, lupus erythematosus, Lymphohistiocytosis, hemophagocytic, Hepatitis A virus cellular receptor 2 gene

Xu Jiaosheng, Fu Libing, Xu Zhe, and Xu Zigang. Subcutaneous panniculitis?like T cell lymphoma[J]. Chinese Journal of Dermatology,2024,e0230172. doi:10.35541/cjd.20230172

References ［43］

［1］	Gonzalez CL, Medeiros LJ, Braziel RM, et al. T⁃cell lymphoma involving subcutaneous tissue. A clinicopathologic entity commonly associated with hemophagocytic syndrome［J］. Am J Surg Pathol, 1991,15（1）:17⁃27. doi: 10.1097/00000478⁃1991 01000⁃00002.
［2］	Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms［J］. Blood, 2016,127（20）:2375⁃2390. doi: 10.1182/blood⁃2016⁃01⁃643569.
［3］	Gau JP, Yang CF, Liu JH, et al. Subcutaneous panniculitis⁃like T cell lymphoma: familial aggregation while different response to chemotherapy［J］. Int J Hematol, 2009,89（1）:63⁃65. doi: 10. 1007/s12185⁃008⁃0202⁃4.
［4］	Nguyen MC, Nguyen V, Le H, et al. Subcutaneous panniculitis⁃like T⁃cell lymphomas with homozygous inheritance of HAVCR2 mutations in Vietnamese pedigrees［J］. Pediatr Blood Cancer, 2021,68（12）:e29292. doi: 10.1002/pbc.29292.
［5］	Gayden T, Sepulveda FE, Khuong⁃Quang DA, et al. Germline HAVCR2 mutations altering TIM⁃3 characterize subcutaneous panniculitis⁃like T cell lymphomas with hemophagocytic lymphohistiocytic syndrome［J］. Nat Genet, 2018,50（12）:1650⁃1657. doi: 10.1038/s41588⁃018⁃0251⁃4.
［6］	Polprasert C, Takeuchi Y, Kakiuchi N, et al. Frequent germline mutations of HAVCR2 in sporadic subcutaneous panniculitis⁃like T⁃cell lymphoma［J］. Blood Adv, 2019,3（4）:588⁃595. doi: 10.1182/bloodadvances.2018028340.
［7］	Sonigo G, Battistella M, Beylot⁃Barry M, et al. HAVCR2 mutations are associated with severe hemophagocytic syndrome in subcutaneous panniculitis⁃like T⁃cell lymphoma［J］. Blood, 2020,135（13）:1058⁃1061. doi: 10.1182/blood.2019003811.
［8］	Koh J, Jang I, Mun S, et al. Genetic profiles of subcutaneous panniculitis⁃like T⁃cell lymphoma and clinicopathological impact of HAVCR2 mutations［J］. Blood Adv, 2021,5（20）:3919⁃3930. doi: 10.1182/bloodadvances.2021004562.
［9］	Guitart J, Mangold AR, Martinez⁃Escala ME, et al. Clinical and pathological characteristics and outcomes among patients with subcutaneous panniculitis⁃like T⁃cell lymphoma and related adipotropic lymphoproliferative disorders［J］. JAMA Dermatol, 2022,158（10）:1167⁃1174. doi: 10.1001/jamadermatol.2022.3347.
［10］	Moonla C, Polprasert C, Komvilaisak P, et al. Germline HAVCR2 mutations and their relation to the clinical spectrum of subcutaneous panniculitis⁃like T⁃cell lymphoma and hemophagocytic lymphohistiocytosis: results from a multicenter study and meta⁃analysis［J］. Haematologica, 2023,108（10）:2743⁃2752. doi: 10.3324/haematol.2022.282419.
［11］	LeBlanc RE, Lansigan F. Unraveling subcutaneous panniculitis⁃like T⁃cell lymphoma: an association between subcutaneous panniculitis⁃like T⁃cell lymphoma, autoimmune lymphoproliferative syndrome, and familial hemophagocytic lymphohistiocytosis［J］. J Cutan Pathol, 2021,48（4）:572⁃577. doi: 10.1111/cup.13863.
［12］	徐基昕, 李彦格, 刘炜, 等. 家族性噬血细胞综合征继发皮下脂膜炎样T细胞淋巴瘤一例并文献复习［J］. 白血病·淋巴瘤, 2021,30（11）:670⁃673. doi: 10.3760/cma.j.cn115356⁃20210409⁃00075.
［13］	Li Z, Lu L, Zhou Z, et al. Recurrent mutations in epigenetic modifiers and the PI3K/AKT/mTOR pathway in subcutaneous panniculitis⁃like T⁃cell lymphoma［J］. Br J Haematol, 2018,181（3）:406⁃410. doi: 10.1111/bjh.14611.
［14］	Bashey S, Krathen M, Abdulla F, et al. Romidepsin is effective in subcutaneous panniculitis⁃like T⁃cell lymphoma［J］. J Clin Oncol, 2012,30（24）:e221⁃e225. doi: 10.1200/JCO.2012.41.5976.
［15］	Nemoto Y, Taniguchi A, Kamioka M, et al. Epstein⁃Barr virus⁃infected subcutaneous panniculitis⁃like T⁃cell lymphoma associated with methotrexate treatment［J］. Int J Hematol, 2010,92（2）:364⁃368. doi: 10.1007/s12185⁃010⁃0642⁃5.
［16］	Matsuoka A, Fujii K, Higashi Y, et al. Subcutaneous panniculitis⁃like T⁃cell lymphoma associated with methotrexate treatment［J］. J Dermatol, 2022,49（12）:e430⁃e432. doi: 10.1111/1346⁃8138. 16524.
［17］	Eser B, Altuntas F, Er O, et al. A case of subcutaneous panniculitis⁃like T⁃cell lymphoma with haemophagocytosis developing secondary to chemotherapy［J］. J Eur Acad Dermatol Venereol, 2004,18（6）:713⁃715. doi: 10.1111/j.1468⁃3083.2004. 01043.x.
［18］	Nakamura H, Sugai T, Kato M, et al. Subcutaneous panniculitis⁃like T⁃cell lymphoma with haemophagocytic syndrome during tocilizumab therapy for juvenile idiopathic arthritis［J］. Clin Exp Rheumatol, 2017,35（1）:174.
［19］	Sluzevich JC, Hall MR, Roy V. Subcutaneous panniculitis⁃like T⁃cell lymphoma after rituximab［J］. J Am Acad Dermatol, 2012,67（5）:e223⁃e225. doi: 10.1016/j.jaad.2012.04.023.
［20］	Hahtola S, Burghart E, Jeskanen L, et al. Clinicopathological characterization and genomic aberrations in subcutaneous panniculitis⁃like T⁃cell lymphoma［J］. J Invest Dermatol, 2008,128（9）:2304⁃2309. doi: 10.1038/jid.2008.6.
［21］	Mangan MS, Sarici AM, Yalcin O, et al. Orbital mass as the only presenting sign with overlapping features of lupus erythematosus panniculitis and subcutaneous panniculitis⁃like T⁃cell lymphoma［J］. Ocul Immunol Inflamm, 2023,31（4）:717⁃720. doi: 10.1080/09273948.2022.2054430.
［22］	Bosisio F, Boi S, Caputo V, et al. Lobular panniculitic infiltrates with overlapping histopathologic features of lupus panniculitis （lupus profundus） and subcutaneous T⁃cell lymphoma: a conceptual and practical dilemma［J］. Am J Surg Pathol, 2015,39（2）:206⁃211. doi: 10.1097/PAS.0000000000000307.
［23］	Wu X, Subtil A, Craiglow B, et al. The coexistence of lupus erythematosus panniculitis and subcutaneous panniculitis⁃like T⁃cell lymphoma in the same patient［J］. JAAD Case Rep, 2018,4（2）:179⁃184. doi: 10.1016/j.jdcr.2017.08.021.
［24］	Tawanwongsri W, Thongsroy J. Subcutaneous panniculitis⁃like T⁃cell lymphoma with a transformation to lupus erythematosus panniculitis: a case report［J］. Case Rep Dermatol, 2022,14（3）:319⁃325. doi: 10.1159/000527530.
［25］	Machan S, Rodríguez M, Alonso⁃Alonso R, et al. Subcutaneous panniculitis⁃like T⁃cell lymphoma, lupus erythematosus profundus, and overlapping cases: molecular characterization through the study of 208 genes［J］. Leuk Lymphoma, 2021,62（9）:2130⁃2140. doi: 10.1080/10428194.2021.1901098.
［26］	de Mingo Pulido Á, Hänggi K, Celias DP, et al. The inhibitory receptor TIM⁃3 limits activation of the cGAS⁃STING pathway in intra⁃tumoral dendritic cells by suppressing extracellular DNA uptake［J］. Immunity, 2021,54（6）:1154⁃1167. doi: 10.1016/j.immuni.2021.04.019.
［27］	Zhang Y, Wang Z, Hu G, et al. A novel germline HAVCR2 （TIM⁃3） compound heterozygous mutation is related to hemophagocytic lymphohistiocytic syndrome in EBV⁃positive peripheral T⁃cell lymphoma （NOS） with down⁃regulated TIM⁃3 signaling［J］. Front Oncol, 2022,12:870676. doi: 10.3389/fonc.2022.870676.
［28］	Maliniemi P, Hahtola S, Ovaska K, et al. Molecular characterization of subcutaneous panniculitis⁃like T⁃cell lymphoma reveals upregulation of immunosuppression⁃ and autoimmunity⁃associated genes［J］. Orphanet J Rare Dis, 2014,9:160. doi: 10.1186/s13023⁃014⁃0160⁃2.
［29］	Honda Y, Otsuka A, Nonomura Y, et al. CCR5 and CXCR3 expression in a case of subcutaneous panniculitis⁃like T⁃cell lymphoma［J］. J Eur Acad Dermatol Venereol, 2016,30（8）:1413⁃1415. doi: 10.1111/jdv.13258.
［30］	Li Z, Wang H, Dong R, et al. Single⁃cell RNA⁃seq reveals characteristics of malignant cells and immune microenvironment in subcutaneous panniculitis⁃like T⁃cell lymphoma［J］. Front Oncol, 2021,11:611580. doi: 10.3389/fonc.2021.611580.
［31］	Liau JY, Chuang SS, Chu CY, et al. The presence of clusters of plasmacytoid dendritic cells is a helpful feature for differentiating lupus panniculitis from subcutaneous panniculitis⁃like T⁃cell lymphoma［J］. Histopathology, 2013,62（7）:1057⁃1066. doi: 10.1111/his.12105.
［32］	López⁃Lerma I, Peñate Y, Gallardo F, et al. Subcutaneous panniculitis⁃like T⁃cell lymphoma: clinical features, therapeutic approach, and outcome in a case series of 16 patients［J］. J Am Acad Dermatol, 2018,79（5）:892⁃898. doi: 10.1016/j.jaad.2018. 05.1243.
［33］	Duan Y, Gao H, Zhou C, et al. A retrospective study of 18 children with subcutaneous panniculitis⁃like T⁃cell lymphoma: multidrug combination chemotherapy or immunomodulatory therapy?［J］. Orphanet J Rare Dis, 2022,17（1）:432. doi: 10. 1186/s13023⁃022⁃02575⁃4.
［34］	Michonneau D, Petrella T, Ortonne N, et al. Subcutaneous panniculitis⁃like T⁃cell lymphoma: immunosuppressive drugs induce better response than polychemotherapy［J］. Acta Derm Venereol, 2017,97（3）:358⁃364. doi: 10.2340/00015555⁃2543.
［35］	Lee WS, Hwang JH, Kim MJ, et al. Cyclosporine A as a primary treatment for panniculitis⁃like T cell lymphoma: a case with a long⁃term remission［J］. Cancer Res Treat, 2014,46（3）:312⁃316. doi: 10.4143/crt.2014.46.3.312.
［36］	Heyman B, Beaven A. Mycophenolate mofetil for the treatment of subcutaneous panniculitis⁃like T⁃cell lymphoma: case report and review of the literature［J］. Clin Lymphoma Myeloma Leuk, 2018,18（10）:e437⁃e440. doi: 10.1016/j.clml.2018.07.283.
［37］	Dietrich C, Keller A, Enk A, et al. Subcutaneous panniculitis⁃like T⁃cell lymphoma: brief review and report of successful treatment with mycophenolate mofetil［J］. Clin Exp Dermatol, 2022,47（7）:1360⁃1363. doi: 10.1111/ced.15145.
［38］	Mehta N, Wayne AS, Kim YH, et al. Bexarotene is active against subcutaneous panniculitis⁃like T⁃cell lymphoma in adult and pediatric populations［J］. Clin Lymphoma Myeloma Leuk, 2012,12（1）:20⁃25. doi: 10.1016/j.clml.2011.06.016.
［39］	Watson LR, Lew TE, Fox LC, et al. Ruxolitinib bridging therapy to allogeneic SCT for high⁃risk refractory subcutaneous panniculitis⁃like T⁃cell lymphoma［J］. Leuk Lymphoma, 2022,63（13）:3217⁃3221. doi: 10.1080/10428194.2022.2118537.
［40］	Hansen S, Alduaij W, Biggs CM, et al. Ruxolitinib as adjunctive therapy for secondary hemophagocytic lymphohistiocytosis: a case series［J］. Eur J Haematol, 2021,106（5）:654⁃661. doi: 10. 1111/ejh.13593.
［41］	曲双, 廖丽昇, 谢颖, 等. PD⁃1单抗联合化疗治疗皮下脂膜炎样T细胞淋巴瘤一例报告并文献复习［J］. 中华血液学杂志, 2021,42（11）:959⁃961. doi: 10.3760/cma.j.issn.0253⁃2727. 2021.11.013.
［42］	Jothishankar B, Espinosa ML, Zain J, et al. Complete response to romidepsin as monotherapy in treatment⁃resistant subcutaneous panniculitis⁃like T⁃cell lymphoma［J］. JAAD Case Rep, 2020,6（12）:1245⁃1247. doi: 10.1016/j.jdcr.2020.09.018.
［43］	Willemze R, Jansen PM, Cerroni L, et al. Subcutaneous panniculitis⁃like T⁃cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases［J］. Blood, 2008,111（2）:838⁃845. doi: 10. 1182/blood⁃2007⁃04⁃087288.

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Subcutaneous panniculitis?like T cell lymphoma

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