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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
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
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

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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