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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

The prognostic and therapeutic potentials of CTLA-4 in hematological malignancies.
Sadeghi M, Khodakarami A, Ahmadi A, Fathi M, Gholizadeh Navashenaq J, Mohammadi H, Yousefi M, Hojjat-Farsangi M, Movasaghpour Akbari AA, Jadidi-Niaragh F
Expert Opin Ther Targets 2022 Dec;26(12):1057-1071. Epub 2023 Jan 24 doi: 10.1080/14728222.2022.2170781. PMID: 36683579
Epidemiology and prognostic factors for mycosis fungoides and Sézary syndrome in a multi-ethnic Asian cohort: a 12-year review.
Lim HLJ, Tan EST, Tee SI, Ho ZY, Boey JJJ, Tan WP, Tang MBY, Shen L, Chan YH, Tan SH
J Eur Acad Dermatol Venereol 2019 Aug;33(8):1513-1521. Epub 2019 Mar 27 doi: 10.1111/jdv.15526. PMID: 30801779
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
Mycosis fungoides and the Sézary syndrome: pathology, staging, and treatment.
Hoppe RT, Wood GS, Abel EA
Curr Probl Cancer 1990 Nov-Dec;14(6):293-371. doi: 10.1016/0147-0272(90)90018-l. PMID: 2245651
Pre-Sézary syndrome.
Winkelmann RK, Buechner SA, Diaz-Perez JL
J Am Acad Dermatol 1984 Jun;10(6):992-9. doi: 10.1016/s0190-9622(84)80321-7. PMID: 6234331

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
Epidemiology and prognostic factors for mycosis fungoides and Sézary syndrome in a multi-ethnic Asian cohort: a 12-year review.
Lim HLJ, Tan EST, Tee SI, Ho ZY, Boey JJJ, Tan WP, Tang MBY, Shen L, Chan YH, Tan SH
J Eur Acad Dermatol Venereol 2019 Aug;33(8):1513-1521. Epub 2019 Mar 27 doi: 10.1111/jdv.15526. PMID: 30801779
Epigenomic Analysis of Sézary Syndrome Defines Patterns of Aberrant DNA Methylation and Identifies Diagnostic Markers.
van Doorn R, Slieker RC, Boonk SE, Zoutman WH, Goeman JJ, Bagot M, Michel L, Tensen CP, Willemze R, Heijmans BT, Vermeer MH
J Invest Dermatol 2016 Sep;136(9):1876-1884. Epub 2016 Apr 23 doi: 10.1016/j.jid.2016.03.042. PMID: 27113428
Pre-Sézary syndrome.
Winkelmann RK, Buechner SA, Diaz-Perez JL
J Am Acad Dermatol 1984 Jun;10(6):992-9. doi: 10.1016/s0190-9622(84)80321-7. PMID: 6234331

Therapy

The prognostic and therapeutic potentials of CTLA-4 in hematological malignancies.
Sadeghi M, Khodakarami A, Ahmadi A, Fathi M, Gholizadeh Navashenaq J, Mohammadi H, Yousefi M, Hojjat-Farsangi M, Movasaghpour Akbari AA, Jadidi-Niaragh F
Expert Opin Ther Targets 2022 Dec;26(12):1057-1071. Epub 2023 Jan 24 doi: 10.1080/14728222.2022.2170781. PMID: 36683579
Mogamulizumab: An Anti-CC Chemokine Receptor 4 Antibody for T-Cell Lymphomas.
Moore DC, Elmes JB, Shibu PA, Larck C, Park SI
Ann Pharmacother 2020 Apr;54(4):371-379. Epub 2019 Oct 25 doi: 10.1177/1060028019884863. PMID: 31648540
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
Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part II. Prognosis, management, and future directions.
Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C
J Am Acad Dermatol 2014 Feb;70(2):223.e1-17; quiz 240-2. doi: 10.1016/j.jaad.2013.08.033. PMID: 24438970
Pre-Sézary syndrome.
Winkelmann RK, Buechner SA, Diaz-Perez JL
J Am Acad Dermatol 1984 Jun;10(6):992-9. doi: 10.1016/s0190-9622(84)80321-7. PMID: 6234331

Prognosis

The prognostic and therapeutic potentials of CTLA-4 in hematological malignancies.
Sadeghi M, Khodakarami A, Ahmadi A, Fathi M, Gholizadeh Navashenaq J, Mohammadi H, Yousefi M, Hojjat-Farsangi M, Movasaghpour Akbari AA, Jadidi-Niaragh F
Expert Opin Ther Targets 2022 Dec;26(12):1057-1071. Epub 2023 Jan 24 doi: 10.1080/14728222.2022.2170781. PMID: 36683579
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
Epidemiology and prognostic factors for mycosis fungoides and Sézary syndrome in a multi-ethnic Asian cohort: a 12-year review.
Lim HLJ, Tan EST, Tee SI, Ho ZY, Boey JJJ, Tan WP, Tang MBY, Shen L, Chan YH, Tan SH
J Eur Acad Dermatol Venereol 2019 Aug;33(8):1513-1521. Epub 2019 Mar 27 doi: 10.1111/jdv.15526. PMID: 30801779
Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part II. Prognosis, management, and future directions.
Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C
J Am Acad Dermatol 2014 Feb;70(2):223.e1-17; quiz 240-2. doi: 10.1016/j.jaad.2013.08.033. PMID: 24438970
Pre-Sézary syndrome.
Winkelmann RK, Buechner SA, Diaz-Perez JL
J Am Acad Dermatol 1984 Jun;10(6):992-9. doi: 10.1016/s0190-9622(84)80321-7. PMID: 6234331

Clinical prediction guides

Integrated genomic analyses of cutaneous T-cell lymphomas reveal the molecular bases for disease heterogeneity.
Park J, Daniels J, Wartewig T, Ringbloom KG, Martinez-Escala ME, Choi S, Thomas JJ, Doukas PG, Yang J, Snowden C, Law C, Lee Y, Lee K, Zhang Y, Conran C, Tegtmeyer K, Mo SH, Pease DR, Jothishankar B, Kwok PY, Abdulla FR, Pro B, Louissaint A, Boggon TJ, Sosman J, Guitart J, Rao D, Ruland J, Choi J
Blood 2021 Oct 7;138(14):1225-1236. doi: 10.1182/blood.2020009655. PMID: 34115827Free PMC Article
Nonmyeloablative allogeneic transplantation achieves clinical and molecular remission in cutaneous T-cell lymphoma.
Weng WK, Arai S, Rezvani A, Johnston L, Lowsky R, Miklos D, Shizuru J, Muffly L, Meyer E, Negrin RS, Wang E, Almazan T, Million L, Khodadoust M, Li S, Hoppe RT, Kim YH
Blood Adv 2020 Sep 22;4(18):4474-4482. doi: 10.1182/bloodadvances.2020001627. PMID: 32941647Free 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
Transcriptome sequencing in Sezary syndrome identifies Sezary cell and mycosis fungoides-associated lncRNAs and novel transcripts.
Lee CS, Ungewickell A, Bhaduri A, Qu K, Webster DE, Armstrong R, Weng WK, Aros CJ, Mah A, Chen RO, Lin M, Sundram U, Chang HY, Kretz M, Kim YH, Khavari PA
Blood 2012 Oct 18;120(16):3288-97. Epub 2012 Aug 30 doi: 10.1182/blood-2012-04-423061. PMID: 22936659Free PMC Article
Novel and highly recurrent chromosomal alterations in Sézary syndrome.
Vermeer MH, van Doorn R, Dijkman R, Mao X, Whittaker S, van Voorst Vader PC, Gerritsen MJ, Geerts ML, Gellrich S, Söderberg O, Leuchowius KJ, Landegren U, Out-Luiting JJ, Knijnenburg J, Ijszenga M, Szuhai K, Willemze R, Tensen CP
Cancer Res 2008 Apr 15;68(8):2689-98. doi: 10.1158/0008-5472.CAN-07-6398. PMID: 18413736

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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