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Staging & Progression

Classification and staging of Mycosis Fungoides and Sézary Syndrome involves evaluation of 4 disease compartments (TNMB)1

Using TNMB criteria, each compartment is classified according to the extent and type of disease involvement in skin, lymph nodes, viscera, and blood.

CLINICAL STAGE CLASSIFICATIONa
T
Skin		 N
Lymph Nodes		 M
Me­t­a­stasis		 B
Blood
Limited skin involvement IA T1 N0 M0 B0-1
Skin-only disease IB T2 N0 M0 B0-1
IIA T1-2 N1-2 M0 B0-1
Tumor stage IIB T3 N0-2 M0 B0-1
Erythro­dermic stage IIIA T4 N0-2 M0 B0
IIIB T4 N0-2 M0 B1
Sézary Syndro­m­e (SS) IVA1 T1-4 N0-2 M0 B2
SS or Mycosis
Fungoid­es (MF)		 IVA2 T1-4 N3 M0 B0-2
Visceral disease IVB T1-4 N0-3 M1 B0-2
Skin

T1=Limited patches/plaques, <10% of skin surface

T2=Generalized patches/plaques, ≥10% of skin surface

T3=≥1 tumor(s) (≥1 cm in diameter)

T4=Erythroderma, ≥80% of skin surface

Lymph Nodes

N0=No abnormal nodes

N1=Clinically abnormal histopathology, Dutch Grade 1 or NCI-LN0-2

N2=Clinically abnormal histopathology, Dutch Grade 2 or NCI-LN3

N3=Clinically abnormal histopathology, Dutch Grade 3-4 or NCI-LN4

Metastasis

M0=No visceral involvement

M1=Visceral involvement

Blood

B0=No blood involvement (<250 atypical cells/μL of lymphocytes)

B1=Low-level involvement (250-1000 atypical cells/μL of lymphocytes)

B2=High-level involvement (>1000 atypical cells/μL of lymphocytes)

Be vigilant for signs of progression in your patients with Mycosis Fungoides and Sézary Syndrome

About 1 in 3 patients with Mycosis Fungoides may experience progression within skin or to other compartments2,b

Up to 20% of patients with early-stage Mycosis Fungoides may have blood involvement3,c

About 1 in 3 patients with mycosis fungoidesmay experience progression within skin or to other compartments, including blood

More extensive skin involvement is associated with poor outcomes2,b

Overall survival, by T classification2

Chart showing overall survival, by T classification

Adapted from Agar N, Wedgeworth E, Crichton S, et al. Journal of Clinical Oncology, Volume 28, Issue 31, p4730-4739. American Society of Clinical Oncology.

  • The considerable pruritus and pain caused by skin lesions, patches, and plaques of Mycosis Fungoides are associated with increased fatigue, anxiety, and depression3,4
  • These symptoms can have a substantial negative impact on daily function and emotional/social well-being3,4

Blood testing via flow cytometry for suspected MF or SS is important at diagnosis and throughout treatment to monitor disease burden and response to treatment1

  • aBlood, viscera, and lymph node classification for each level can further be characterized by T-cell receptor clone status, with A=clone negative or equivocal and B=clone positive with identical clone in the skin.1,5
  • bBased on a survival outcomes and prognostic factors study that included 1502 patients with Mycosis Fungoides/Sézary Syndrome.2
  • cBased on a PROCLIPI database analysis that included 348 patients with early-stage Mycosis Fungoides.3
  • CCR4=C-C chemokine receptor type 4; NCI-LN=National Cancer Institute - Lymph Nodes grade system; TNMB=tumor-node-metastasis-blood

See how POTELIGEO targets CCR4+
T cells for destruction

Explore MOA
References:
  1. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Primary Cutaneous Lymphomas V.1.2025. © National Comprehensive Cancer Network, Inc. 2024. All rights reserved. Accessed February 11, 2025. To view the most recent and complete version of the guideline, go online to NCCN.org.
  2. 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.
  3. Scarisbrick JJ, Quaglino P, Prince HM, et al. The PROCLIPI international registry of early-stage mycosis fungoides identifies substantial diagnostic delay in most patients. Br J Dermatol. 2019;181(2):350-357.
  4. Ottevanger R, van Beugen S, Evers AWM, Willemze R, Vermeer MH, Quint KD. Quality of life in patients with mycosis fungoides and Sézary syndrome: a systematic review of the literature. J Eur Acad Dermatol Venereol. 2021;35(12):2377-2387.
  5. 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(5):419-437.

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.

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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.