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ABOUT SÉZARY SYNDROME (SS)

Sézary Syndrome is an aggressive cutaneous T-cell lymphoma (CTCL) subtype1

Typical Sézary Syndrome presentation on the skin is highly erythrodermic; Sézary Syndrome also involves a high blood tumor burden and generalized lymphadenopathy2

Blood involvement in Sézary Syndrome:

Is usually classified as B2

  • Sézary Syndrome is characterized by the presence of abnormal (Sézary) cells in the blood2
  • B2 is defined as ≥1000/μL Sézary cells with positive clone or other aberrant phenotype in the presence of a relevant T-cell clone in blood4

Is associated with poorer prognosis/shorter survival for both Mycosis Fungoides and Sézary Syndrome3

  • Patients with B2 blood involvement have a shorter life expectancy and are associated with more negative outcomes as compared to those without blood involvement (B0)
  • There are mixed outcomes associated with patients with B1 blood involvement4

Two studies found a significant difference in median overall survival for B0 and B23,5

Median overall survival (years) for B0 blood classification is 18 years in Agar et al and 29.3 years in Talpur et al
  • In a third study, Scarisbrick et al also found a trend toward worse survival with increasing blood involvement6

National Comprehensive Cancer Network® (NCCN®) currently defines blood classification based on the following8,9:

BLOOD CLASSIFICATION

  • d

  • <250/μL
    CD4+CD26- or CD4+CD7-
    cells by flow cytometry

  • 250/μL to <1000/μL
    CD4+CD26- or CD4+CD7-
    cells by flow cytometry

  • ≥1000/μL
    CD4+CD7- or CD4+CD26-
    cells or other aberrant phenotype in the presence of a relevant T-cell clone in blood

  • NCCN=National Comprehensive Cancer Network
  • aBlood classification defined in the Agar study: B0=No blood involvement (≤5% Sézary cells); B0a=clone-negative; B0b=clone-positive; B1=low tumor burden (>5% Sézary cells but not meeting B2 criteria); B2=high tumor burden (≥1000/μL Sézary cells with positive clone).3,7
  • bRetrospective analysis of patients with Mycosis Fungoides and Sézary Syndrome from the ICARSIS CTCL registry (1980-2009).3
  • cIn the Agar study, B0 represents those patients without T-cell receptor (TCR) data.3
  • dBlood classification defined in the Talpur study: B0=No blood involvement (<500 Sézary cells/μL); B1=low tumor burden (>500 and <1000 cells/μL); B2=high tumor burden (>1000 cells/μL or >35% of lymphocytes as CD4+CD26– or CD4+CD7– cells).5
  • eIn the Talpur study, median survival for patients with B1 levels of blood involvement was not reached due to limited number of patients.5

CTCL Expert Perspectives: Patient Communication

Lauren C. Pinter-Brown, MD, clinical professor of Hematology/Oncology at University of California, Irvine, discusses how to communicate with patients who have Mycosis Fungoides or Sézary Syndrome on topics including disease state, prognosis, blood involvement, staging and prescribing.

Blood testing via flow cytometry for suspected Sézary Syndrome is important at diagnosis and throughout treatment to monitor disease burden and response to treatment10

Explore POTELIGEO MOA
References:
  1. Kim YH, Bagot M, Pinter-Brown L, et al. Mogamulizumab versus vorinostat in previously treated cutaneous T-cell lymphoma (MAVORIC): an international, open-label, randomised, controlled phase 3 trial. Lancet Oncol. 2018;19(9):1192-1204.
  2. Willemze R, Cerroni L, Kempf W, et al. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood. 2019;133(16):1703-1714.
  3. Agar NS, Wedgeworth E, Crichton S, et al. Survival outcomes and prognostic factors in mycosis fungoides/Sézary syndrome: validation of the revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer staging proposal. J Clin Oncol. 2010;28(31):4730-4739.
  4. Scarisbrick JJ, Hodak E, Bagot M, et al. Blood classification and blood response criteria in mycosis fungoides and Sézary syndrome using flow cytometry: recommendations from the EORTC cutaneous lymphoma task force. Eur J Cancer. 2018;93:47-56.
  5. Talpur R, Singh L, Daulat S, et al. Long-term outcomes of 1,263 patients with mycosis fungoides and Sézary syndrome from 1982 to 2009. Clin Cancer Res. 2012;18(18):5051-5060.
  6. Scarisbrick JJ, Princes HM, Vermeer MH, et al. Cutaneous lymphoma international consortium study of outcome in advanced stages of mycosis fungoides and Sézary syndrome: effect of specific prognostic markers on survival and development of a prognostic model. J Clin Oncol. 2015;33(32):3766-3773.
  7. Olsen E, Vonderheid E, Pimpinelli N, et al. Revisions to the staging and classification of mycosis fungoides and Sézary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007;110(6):1713-1722.
  8. Olsen EA, Whittaker S, Willemze R, et al. Primary cutaneous lymphoma: recommendations for clinical trial design and staging update from the ISCL, USCLC, and EORTC. Blood. 2022;140:419-437
  9. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Primary Cutaneous Lymphomas V.1.2023. © National Comprehensive Cancer Network, Inc. 2023. All rights reserved. Accessed July 13, 2023. To view the most recent and complete version of the guideline, go online to NCCN.org.
  10. Vermeer MH, Moins-Teisserenc H, Bagot M, Quaglino P, Whittaker S. Flow cytometry for the assessment of blood tumour burden in cutaneous T-cell lymphoma: towards a standardized approach. Br J Dermatol. 2022;187(1):21-28.

Indication

POTELIGEO® (mogamulizumab-kpkc) injection for intravenous infusion is indicated for the treatment of adult patients with relapsed or refractory mycosis fungoides (MF) or Sézary syndrome (SS) after at least one prior systemic therapy.

Important Safety Information

Warnings and Precautions

Dermatologic toxicity: Monitor patients for rash throughout the course of treatment. For patients who experienced dermatologic toxicity in Trial 1, the median time to onset was 15 weeks, with 25% of cases occurring after 31 weeks. Interrupt POTELIGEO for moderate or severe rash (Grades 2 or 3). Permanently discontinue POTELIGEO for life-threatening (Grade 4) rash or for any Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN).

Infusion reactions: Most infusion reactions occur during or shortly after the first infusion. Infusion reactions can also occur with subsequent infusions. Monitor patients closely for signs and symptoms of infusion reactions and interrupt the infusion for any grade reaction and treat promptly. Permanently discontinue POTELIGEO for any life-threatening (Grade 4) infusion reaction.

Infections: Monitor patients for signs and symptoms of infection and treat promptly.

Autoimmune complications: Interrupt or permanently discontinue POTELIGEO as appropriate for suspected immune-mediated adverse reactions. Consider the benefit/risk of POTELIGEO in patients with a history of autoimmune disease.

Complications of allogeneic HSCT after POTELIGEO: Increased risks of transplant complications have been reported in patients who received allogeneic HSCT after POTELIGEO. Follow patients closely for early evidence of transplant-related complications.

Adverse Reactions

The most common adverse reactions (reported in ≥10% of patients) with POTELIGEO in the clinical trial were rash, including drug eruption (35%), infusion reaction (33%), fatigue (31%), diarrhea (28%), drug eruption (24%), upper respiratory tract infection (22%), musculoskeletal pain (22%), skin infection (19%), pyrexia (17%), edema (16%), nausea (16%), headache (14%), thrombocytopenia (14%), constipation (13%), anemia (12%), mucositis (12%), cough (11%), and hypertension (10%).

You are encouraged to report suspected adverse reactions to Kyowa Kirin, Inc. at 1-844-768-3544 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.

Please see additional Important Safety Information in full Prescribing Information as well as Patient Information.

Indication

POTELIGEO® (mogamulizumab-kpkc) injection for intravenous infusion is indicated for the treatment of adult patients with relapsed or refractory mycosis fungoides (MF) or Sézary syndrome (SS) after at least one prior systemic therapy.

Important Safety Information

Warnings and Precautions

Dermatologic toxicity: Monitor patients for rash throughout the course of treatment. For patients who experienced dermatologic toxicity in Trial 1, the median time to onset was 15 weeks, with 25% of cases occurring after 31 weeks. Interrupt POTELIGEO for moderate or severe rash (Grades 2 or 3). Permanently discontinue POTELIGEO for life-threatening (Grade 4) rash or for any Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN).

Infusion reactions: Most infusion reactions occur during or shortly after the first infusion. Infusion reactions can also occur with subsequent infusions. Monitor patients closely for signs and symptoms of infusion reactions and interrupt the infusion for any grade reaction and treat promptly. Permanently discontinue POTELIGEO for any life-threatening (Grade 4) infusion reaction.

Infections: Monitor patients for signs and symptoms of infection and treat promptly.

Autoimmune complications: Interrupt or permanently discontinue POTELIGEO as appropriate for suspected immune-mediated adverse reactions. Consider the benefit/risk of POTELIGEO in patients with a history of autoimmune disease.

Complications of allogeneic HSCT after POTELIGEO: Increased risks of transplant complications have been reported in patients who received allogeneic HSCT after POTELIGEO. Follow patients closely for early evidence of transplant-related complications.

Adverse Reactions

The most common adverse reactions (reported in ≥10% of patients) with POTELIGEO in the clinical trial were rash, including drug eruption (35%), infusion reaction (33%), fatigue (31%), diarrhea (28%), drug eruption (24%), upper respiratory tract infection (22%), musculoskeletal pain (22%), skin infection (19%), pyrexia (17%), edema (16%), nausea (16%), headache (14%), thrombocytopenia (14%), constipation (13%), anemia (12%), mucositis (12%), cough (11%), and hypertension (10%).

You are encouraged to report suspected adverse reactions to Kyowa Kirin, Inc. at 1-844-768-3544 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.

Please see additional Important Safety Information in full Prescribing Information as well as Patient Information.