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Recurrent Sezary Syndrome

MedGen UID:
1778271
•Concept ID:
C0862201
•
Neoplastic Process

Definition

The reemergence of Sezary syndrome after a period of remission. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRecurrent Sezary Syndrome

Professional guidelines

PubMed

Clinical Characterization of Mogamulizumab-Associated Rash During Treatment of Mycosis Fungoides or Sézary Syndrome.
Hirotsu KE, Neal TM, Khodadoust MS, Wang JY, Rieger KE, Strelo J, Hong E, Kim YH, Kwong BY
JAMA Dermatol 2021 Jun 1;157(6):700-707. doi: 10.1001/jamadermatol.2021.0877. PMID: 33881447Free PMC Article
Brentuximab vedotin for treatment of non-Hodgkin lymphomas: A systematic review.
Berger GK, McBride A, Lawson S, Royball K, Yun S, Gee K, Bin Riaz I, Saleh AA, Puvvada S, Anwer F
Crit Rev Oncol Hematol 2017 Jan;109:42-50. Epub 2016 Nov 21 doi: 10.1016/j.critrevonc.2016.11.009. PMID: 28010897Free PMC Article
Panoptic clinical review of the current and future treatment of relapsed/refractory T-cell lymphomas: Cutaneous T-cell lymphomas.
Zinzani PL, Bonthapally V, Huebner D, Lutes R, Chi A, Pileri S
Crit Rev Oncol Hematol 2016 Mar;99:228-40. Epub 2016 Jan 2 doi: 10.1016/j.critrevonc.2015.12.018. PMID: 26811014

Recent clinical studies

Etiology

Mogamulizumab versus vorinostat in previously treated cutaneous T-cell lymphoma (MAVORIC): an international, open-label, randomised, controlled phase 3 trial.
Kim YH, Bagot M, Pinter-Brown L, Rook AH, Porcu P, Horwitz SM, Whittaker S, Tokura Y, Vermeer M, Zinzani PL, Sokol L, Morris S, Kim EJ, Ortiz-Romero PL, Eradat H, Scarisbrick J, Tsianakas A, Elmets C, Dalle S, Fisher DC, Halwani A, Poligone B, Greer J, Fierro MT, Khot A, Moskowitz AJ, Musiek A, Shustov A, Pro B, Geskin LJ, Dwyer K, Moriya J, Leoni M, Humphrey JS, Hudgens S, Grebennik DO, Tobinai K, Duvic M; MAVORIC Investigators
Lancet Oncol 2018 Sep;19(9):1192-1204. Epub 2018 Aug 9 doi: 10.1016/S1470-2045(18)30379-6. PMID: 30100375
Mature T-cell leukemias: Molecular and Clinical Aspects.
Bailey NG, Elenitoba-Johnson KS
Curr Hematol Malig Rep 2015 Dec;10(4):421-8. doi: 10.1007/s11899-015-0288-8. PMID: 26433891
Pralatrexate alone or in combination with bexarotene: long-term tolerability in relapsed/refractory mycosis fungoides.
Talpur R, Thompson A, Gangar P, Duvic M
Clin Lymphoma Myeloma Leuk 2014 Aug;14(4):297-304. Epub 2014 Feb 4 doi: 10.1016/j.clml.2014.01.010. PMID: 24589156
Comprehensive analysis of PTEN status in Sezary syndrome.
Cristofoletti C, Picchio MC, Lazzeri C, Tocco V, Pagani E, Bresin A, Mancini B, Passarelli F, Facchiano A, Scala E, Lombardo GA, Cantonetti M, Caprini E, Russo G, Narducci MG
Blood 2013 Nov 14;122(20):3511-20. Epub 2013 Sep 23 doi: 10.1182/blood-2013-06-510578. PMID: 24062018
Photopheresis therapy for cutaneous T-cell lymphoma.
Duvic M, Hester JP, Lemak NA
J Am Acad Dermatol 1996 Oct;35(4):573-9. doi: 10.1016/s0190-9622(96)90683-0. PMID: 8859287

Diagnosis

Cutaneous T-cell lymphoma: diagnosing subtypes and the challenges.
Hague C, Farquharson N, Menasce L, Parry E, Cowan R
Br J Hosp Med (Lond) 2022 Apr 2;83(4):1-7. Epub 2022 Apr 14 doi: 10.12968/hmed.2021.0149. PMID: 35506718
T-cell and NK-cell lymphomas in the lung.
Pan Z, Xu ML
Semin Diagn Pathol 2020 Nov;37(6):273-282. Epub 2020 May 16 doi: 10.1053/j.semdp.2020.04.003. PMID: 32448591
Sézary syndrome managed with histone deacetylase inhibitor followed by anti-CCR4 monoclonal antibody.
Numata T, Nagatani T, Shirai K, Maeda T, Mae K, Nakasu M, Saito M, Usuda T, Tsuboi R, Okubo Y
Clin Exp Dermatol 2018 Apr;43(3):281-285. Epub 2018 Jan 12 doi: 10.1111/ced.13357. PMID: 29327377
Treatment of cutaneous lymphoma with etretinate.
Claudy AL, Rouchouse B, Boucheron S, Le Petit JC
Br J Dermatol 1983 Jul;109(1):49-56. doi: 10.1111/j.1365-2133.1983.tb03991.x. PMID: 6860571
Lymphomatoid contact dermatitis.
Ecker RI, Winkelmann RK
Contact Dermatitis 1981 Mar;7(2):84-93. doi: 10.1111/j.1600-0536.1981.tb03985.x. PMID: 7238017

Therapy

Spectrum of mutational signatures in T-cell lymphoma reveals a key role for UV radiation in cutaneous T-cell lymphoma.
Jones CL, Degasperi A, Grandi V, Amarante TD; Genomics England Research Consortium, Mitchell TJ, Nik-Zainal S, Whittaker SJ
Sci Rep 2021 Feb 17;11(1):3962. doi: 10.1038/s41598-021-83352-4. PMID: 33597573Free PMC Article
Mogamulizumab versus vorinostat in previously treated cutaneous T-cell lymphoma (MAVORIC): an international, open-label, randomised, controlled phase 3 trial.
Kim YH, Bagot M, Pinter-Brown L, Rook AH, Porcu P, Horwitz SM, Whittaker S, Tokura Y, Vermeer M, Zinzani PL, Sokol L, Morris S, Kim EJ, Ortiz-Romero PL, Eradat H, Scarisbrick J, Tsianakas A, Elmets C, Dalle S, Fisher DC, Halwani A, Poligone B, Greer J, Fierro MT, Khot A, Moskowitz AJ, Musiek A, Shustov A, Pro B, Geskin LJ, Dwyer K, Moriya J, Leoni M, Humphrey JS, Hudgens S, Grebennik DO, Tobinai K, Duvic M; MAVORIC Investigators
Lancet Oncol 2018 Sep;19(9):1192-1204. Epub 2018 Aug 9 doi: 10.1016/S1470-2045(18)30379-6. PMID: 30100375
Sézary syndrome managed with histone deacetylase inhibitor followed by anti-CCR4 monoclonal antibody.
Numata T, Nagatani T, Shirai K, Maeda T, Mae K, Nakasu M, Saito M, Usuda T, Tsuboi R, Okubo Y
Clin Exp Dermatol 2018 Apr;43(3):281-285. Epub 2018 Jan 12 doi: 10.1111/ced.13357. PMID: 29327377
Romidepsin for cutaneous T-cell lymphoma.
Prince HM, Dickinson M, Khot A
Future Oncol 2013 Dec;9(12):1819-27. doi: 10.2217/fon.13.220. PMID: 24295412
Treatment of cutaneous lymphoma with etretinate.
Claudy AL, Rouchouse B, Boucheron S, Le Petit JC
Br J Dermatol 1983 Jul;109(1):49-56. doi: 10.1111/j.1365-2133.1983.tb03991.x. PMID: 6860571

Prognosis

Cutaneous T-cell lymphoma: diagnosing subtypes and the challenges.
Hague C, Farquharson N, Menasce L, Parry E, Cowan R
Br J Hosp Med (Lond) 2022 Apr 2;83(4):1-7. Epub 2022 Apr 14 doi: 10.12968/hmed.2021.0149. PMID: 35506718
Discussion on the indication of allogeneic stem cell transplantation for advanced cutaneous T cell lymphomas.
Novelli S, Monter A, Pilar García-Muret M, Martino R, Briones J, Sierra J
Int J Hematol 2019 Oct;110(4):406-410. Epub 2019 Jul 17 doi: 10.1007/s12185-019-02707-w. PMID: 31317515
Mature T-cell leukemias: Molecular and Clinical Aspects.
Bailey NG, Elenitoba-Johnson KS
Curr Hematol Malig Rep 2015 Dec;10(4):421-8. doi: 10.1007/s11899-015-0288-8. PMID: 26433891
Comprehensive analysis of PTEN status in Sezary syndrome.
Cristofoletti C, Picchio MC, Lazzeri C, Tocco V, Pagani E, Bresin A, Mancini B, Passarelli F, Facchiano A, Scala E, Lombardo GA, Cantonetti M, Caprini E, Russo G, Narducci MG
Blood 2013 Nov 14;122(20):3511-20. Epub 2013 Sep 23 doi: 10.1182/blood-2013-06-510578. PMID: 24062018
Lymphomatoid contact dermatitis.
Ecker RI, Winkelmann RK
Contact Dermatitis 1981 Mar;7(2):84-93. doi: 10.1111/j.1600-0536.1981.tb03985.x. PMID: 7238017

Clinical prediction guides

Spectrum of mutational signatures in T-cell lymphoma reveals a key role for UV radiation in cutaneous T-cell lymphoma.
Jones CL, Degasperi A, Grandi V, Amarante TD; Genomics England Research Consortium, Mitchell TJ, Nik-Zainal S, Whittaker SJ
Sci Rep 2021 Feb 17;11(1):3962. doi: 10.1038/s41598-021-83352-4. PMID: 33597573Free PMC Article
Mogamulizumab versus vorinostat in previously treated cutaneous T-cell lymphoma (MAVORIC): an international, open-label, randomised, controlled phase 3 trial.
Kim YH, Bagot M, Pinter-Brown L, Rook AH, Porcu P, Horwitz SM, Whittaker S, Tokura Y, Vermeer M, Zinzani PL, Sokol L, Morris S, Kim EJ, Ortiz-Romero PL, Eradat H, Scarisbrick J, Tsianakas A, Elmets C, Dalle S, Fisher DC, Halwani A, Poligone B, Greer J, Fierro MT, Khot A, Moskowitz AJ, Musiek A, Shustov A, Pro B, Geskin LJ, Dwyer K, Moriya J, Leoni M, Humphrey JS, Hudgens S, Grebennik DO, Tobinai K, Duvic M; MAVORIC Investigators
Lancet Oncol 2018 Sep;19(9):1192-1204. Epub 2018 Aug 9 doi: 10.1016/S1470-2045(18)30379-6. PMID: 30100375
Array-CGH analysis of cutaneous anaplastic large cell lymphoma.
Szuhai K, van Doorn R, Tensen CP, Van Kester
Methods Mol Biol 2013;973:197-212. doi: 10.1007/978-1-62703-281-0_13. PMID: 23412792
Photopheresis therapy for cutaneous T-cell lymphoma.
Duvic M, Hester JP, Lemak NA
J Am Acad Dermatol 1996 Oct;35(4):573-9. doi: 10.1016/s0190-9622(96)90683-0. PMID: 8859287
PUVA treatment of erythrodermic and plaque-type mycosis fungoides. Ten-year follow-up study.
Abel EA, Sendagorta E, Hoppe RT, Hu CH
Arch Dermatol 1987 Jul;123(7):897-901. PMID: 3606168

Recent systematic reviews

Allogeneic stem cell transplant for treatment of mycosis fungoides and Sezary syndrome: a systematic review and meta-analysis.
Goyal A, O'Leary D, Foss F
Bone Marrow Transplant 2024 Jan;59(1):41-51. Epub 2023 Oct 18 doi: 10.1038/s41409-023-02122-0. PMID: 37853164
Efficacy of Allogeneic Hematopoietic Cell Transplantation in Cutaneous T Cell Lymphoma: Results of a Systematic Review and Meta-Analysis.
Iqbal M, Reljic T, Ayala E, Sher T, Murthy H, Roy V, Foran J, Tun H, Kumar A, Kharfan-Dabaja MA
Biol Blood Marrow Transplant 2020 Jan;26(1):76-82. Epub 2019 Sep 5 doi: 10.1016/j.bbmt.2019.08.019. PMID: 31494227
Comparison of Narrowband UV-B With Psoralen-UV-A Phototherapy for Patients With Early-Stage Mycosis Fungoides: A Systematic Review and Meta-analysis.
Phan K, Ramachandran V, Fassihi H, Sebaratnam DF
JAMA Dermatol 2019 Mar 1;155(3):335-341. doi: 10.1001/jamadermatol.2018.5204. PMID: 30698622Free PMC Article
Brentuximab vedotin for treatment of non-Hodgkin lymphomas: A systematic review.
Berger GK, McBride A, Lawson S, Royball K, Yun S, Gee K, Bin Riaz I, Saleh AA, Puvvada S, Anwer F
Crit Rev Oncol Hematol 2017 Jan;109:42-50. Epub 2016 Nov 21 doi: 10.1016/j.critrevonc.2016.11.009. PMID: 28010897Free PMC Article
Molecular cytogenetic characterization of Sézary syndrome.
Mao X, Lillington DM, Czepulkowski B, Russell-Jones R, Young BD, Whittaker S
Genes Chromosomes Cancer 2003 Mar;36(3):250-60. doi: 10.1002/gcc.10152. PMID: 12557225

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