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Refractory Sezary Syndrome

MedGen UID:
1788498
•Concept ID:
C0862207
•
Neoplastic Process

Definition

Sezary syndrome that is resistant to treatment. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Pralatrexate and total skin electron beam therapy as bridging agents to allogeneic stem cell transplantation in severely treatment-refractory Sezary syndrome.
Truong K, Bhattacharyya A, Smee R, Kim J, Wells J
Int J Dermatol 2023 Apr;62(4):e219-e221. Epub 2023 Jan 22 doi: 10.1111/ijd.16587. PMID: 36683181
Alemtuzumab is an effective third-line treatment versus single-agent gemcitabine or pralatrexate for refractory Sézary syndrome: a systematic review.
Stewart JR, Desai N, Rizvi S, Zhu H, Goff HW
Eur J Dermatol 2018 Dec 1;28(6):764-774. doi: 10.1684/ejd.2018.3444. PMID: 30591425

Recent clinical studies

Etiology

Brentuximab Vedotin for Relapsed or Refractory Sézary Syndrome.
Lewis DJ, Haun PL, Samimi SS, Vittorio CC, Villasenor-Park J, Barta SK, Landsburg DJ, Svoboda J, Nasta SD, Schuster SJ, Rook AH, Kim EJ
JAMA Dermatol 2021 Mar 1;157(3):317-321. doi: 10.1001/jamadermatol.2020.4901. PMID: 33377934Free PMC Article
Alemtuzumab is an effective third-line treatment versus single-agent gemcitabine or pralatrexate for refractory Sézary syndrome: a systematic review.
Stewart JR, Desai N, Rizvi S, Zhu H, Goff HW
Eur J Dermatol 2018 Dec 1;28(6):764-774. doi: 10.1684/ejd.2018.3444. PMID: 30591425
Durable clinical, cytogenetic, and molecular remissions after allogeneic hematopoietic cell transplantation for refractory Sezary syndrome and mycosis fungoides.
Molina A, Zain J, Arber DA, Angelopolou M, O'Donnell M, Murata-Collins J, Forman SJ, Nademanee A
J Clin Oncol 2005 Sep 1;23(25):6163-71. doi: 10.1200/JCO.2005.02.774. PMID: 16135483

Diagnosis

Brentuximab Vedotin for Relapsed or Refractory Sézary Syndrome.
Lewis DJ, Haun PL, Samimi SS, Vittorio CC, Villasenor-Park J, Barta SK, Landsburg DJ, Svoboda J, Nasta SD, Schuster SJ, Rook AH, Kim EJ
JAMA Dermatol 2021 Mar 1;157(3):317-321. doi: 10.1001/jamadermatol.2020.4901. PMID: 33377934Free PMC Article
Successful treatment of chemotherapy-refractory Sézary syndrome with alemtuzumab (Campath-1H).
Gautschi O, Blumenthal N, Streit M, Solenthaler M, Hunziker T, Zenhäusern R
Eur J Haematol 2004 Jan;72(1):61-3. doi: 10.1046/j.0902-4441.2004.00169.x. PMID: 14962265
Remission of refractory Sezary syndrome after bone marrow transplantation from a matched unrelated donor.
Molina A, Nademanee A, Arber DA, Forman SJ
Biol Blood Marrow Transplant 1999;5(6):400-4. doi: 10.1016/s1083-8791(99)70017-0. PMID: 10595818

Therapy

Brentuximab Vedotin for Relapsed or Refractory Sézary Syndrome.
Lewis DJ, Haun PL, Samimi SS, Vittorio CC, Villasenor-Park J, Barta SK, Landsburg DJ, Svoboda J, Nasta SD, Schuster SJ, Rook AH, Kim EJ
JAMA Dermatol 2021 Mar 1;157(3):317-321. doi: 10.1001/jamadermatol.2020.4901. PMID: 33377934Free PMC Article
Alemtuzumab is an effective third-line treatment versus single-agent gemcitabine or pralatrexate for refractory Sézary syndrome: a systematic review.
Stewart JR, Desai N, Rizvi S, Zhu H, Goff HW
Eur J Dermatol 2018 Dec 1;28(6):764-774. doi: 10.1684/ejd.2018.3444. PMID: 30591425
Alemtuzumab in refractory Sézary syndrome.
Reifs CM, Salido-Vallejo R, Garnacho-Saucedo GM, Corte-Sánchez S, González-Menchen A, García-Nieto AV
An Bras Dermatol 2016 Sep-Oct;91(5):642-644. doi: 10.1590/abd1806-4841.20164322. PMID: 27828640Free PMC Article
A case of refractory Sézary syndrome with large-cell transformation responsive to brentuximab vedotin.
Corey K, Cook D, Bekker J, Mugnaini E, Lin JH
JAMA Dermatol 2014 Feb;150(2):210-2. doi: 10.1001/jamadermatol.2013.5741. PMID: 24352253
Bexarotene monotherapy for patients with refractory Sézary syndrome.
Staib G, Scharffetter-Kochanek K
Dermatol Clin 2008 Jan;26 Suppl 1:61-3. PMID: 18405190

Prognosis

Complete Response to tenalisib and romidepsin with long-term maintenance using tenalisib monotherapy in a patient with relapsed and refractory sézary syndrome.
Nguyen CN, Iyer SP, Duvic M, Barde PJ, Nair A, Routhu KV, Huen AO
Invest New Drugs 2023 Apr;41(2):350-355. Epub 2023 Apr 1 doi: 10.1007/s10637-022-01315-6. PMID: 37004644
Brentuximab Vedotin for Relapsed or Refractory Sézary Syndrome.
Lewis DJ, Haun PL, Samimi SS, Vittorio CC, Villasenor-Park J, Barta SK, Landsburg DJ, Svoboda J, Nasta SD, Schuster SJ, Rook AH, Kim EJ
JAMA Dermatol 2021 Mar 1;157(3):317-321. doi: 10.1001/jamadermatol.2020.4901. PMID: 33377934Free PMC Article
A novel regimen of vorinostat with interferon gamma for refractory Sézary syndrome.
Gardner JM, Introcaso CE, Nasta SD, Kim EJ, Vittorio CC, Rook AH
J Am Acad Dermatol 2009 Jul;61(1):112-6. doi: 10.1016/j.jaad.2008.11.889. PMID: 19539845
Fatal adenoviral and enteroviral infections and an Epstein-Barr virus positive large B-cell lymphoma after alemtuzumab treatment in a patient with refractory Sézary syndrome.
Roch N, Salameire D, Gressin R, Morand P, Epaulard O, Pavese P, Brion JP, Stahl JP
Scand J Infect Dis 2008;40(4):343-6. doi: 10.1080/00365540701684817. PMID: 17934981

Clinical prediction guides

Deoxycoformycin in therapy of refractory lymphoid neoplasms.
Ho AD, Thaler J, Kuse R, Willemze R, Mandelli F, Lauria F, Zittoun R, Stryckmans P
Onkologie 1988 Feb;11(1):35-7. doi: 10.1159/000216477. PMID: 3283623

Recent systematic reviews

Alemtuzumab is an effective third-line treatment versus single-agent gemcitabine or pralatrexate for refractory Sézary syndrome: a systematic review.
Stewart JR, Desai N, Rizvi S, Zhu H, Goff HW
Eur J Dermatol 2018 Dec 1;28(6):764-774. doi: 10.1684/ejd.2018.3444. PMID: 30591425

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