Prescribing Extracorporeal Photopheresis (ECP) to Treat CTCL Skin Symptoms in Patients Unresponsive to Other Forms of Treatment
Identifying Potential Patients
Presenting with skin manifestations of CTCL unresponsive to other forms of treatment.1
The most common forms of CTCL are mycosis fungoides, which accounts for ~60% of cases, and Sézary syndrome, which accounts for 5% of cases.2 Skin symptoms of mycosis fungoides typically have a long, indolent course.3,4
Mycosis fungoides
It is characterized by patches or plaques on the skin, which can mimic benign skin conditions (eg, eczema, dermatitis, or psoriasis).3 Early stages of mycosis fungoides are often managed with skin-directed treatments, whereas unresolved skin manifestations may require systemic treatments.5
Sézary syndrome
A leukemic variant with a generally poor prognosis. These patients typically have exfoliating skin, edema, and lichenification, with potentially severe fissuring, scaling, and itching. Patients with Sézary syndrome may also present with erythroderma (red man syndrome) and/or enlarged lymph nodes.6,7
Patients may experience a long series of different treatments5,8
Most patients with skin manifestations of CTCL experience slower progression of their underlying disease than patients with other forms of non-Hodgkin lymphoma (NHL) and should be treated accordingly.6,9,10 Given the chronic nature of CTCL, tolerability is an important consideration when choosing treatments.8
Evaluate Response and Adjust Treatment Schedule if Needed
Evaluate the response
- The normal treatment schedule is 2 consecutive days every 4 weeks for a minimum of 7 treatment cycles (6 months)1
- Assess the patient at ~3 months (during the 4th treatment cycle) to determine skin score change from baseline1
- The 3-month patient assessment can help inform whether to adjust the treatment schedule if indicated1
Adjust the schedule if indicated
- For patients whose skin score has increased from baseline, consider increasing treatment frequency to 2 consecutive treatments every 2 weeks, which is the accelerated treatment schedule1
- If a 25% improvement in the skin score is attained after 4 consecutive weeks, the regular treatment schedule may resume1
- There is no clinical evidence to show that treatment with UVADEX® (methoxsalen) beyond 6 months or using a different schedule provides additional benefit1
Explain your rationale
- Patients in the clinical study who were unresponsive to the normal treatment schedule were then placed on the accelerated schedule and reassessed for response1
- Communicating this type of information may help reduce patients’ anxiety, manage expectations, and increase involvement in their own care11
Prepare Your Patients by Setting Expectations
Help them know before they go
Patient adherence to medical recommendations may be impacted through the use of a patient-centered communication style to help build rapport and establish goals. Presenting patients with factual and clear recommendations can help patients follow their treatment plan.12
Photopheresis is not an immediate-response treatment
Clinical response to immunotherapy may take longer to assess than with conventional agents because immunotherapy works by triggering an immune response.13 Set expectations that results may come, but it could take at least 3 to 6 months.14
An assessment is appropriate during the fourth treatment cycle, which is approximately 3 months after initiation. The approved course of treatment is a minimum of 7 cycles.1
These minor lifestyle adjustments may help patients get ready
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Hydrating prior to the procedure in anticipation of fluid shifts.15,16 Drinking enough water and juice while reducing alcohol and caffeine intake starting 2 days prior to a procedure is preferred17,18
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Avoiding high-fat foods the night before and on the day of the procedure.15 Low-fat, healthy meals are preferred
Patient information and resources
Connecting Patients to Treatment Centers*
Find a local treatment center or contact our Customer Care team at 877-566-9466.*Treatment centers are independent, third-party facilities not owned or operated by Mallinckrodt Pharmaceuticals.
Patient stories
It may help your patients to hear from people who were treated with THERAKOS® Photopheresis. There are a number of personal stories available that cover a range of experiences that your patients may find relatable.
References
1. UVADEX (methoxsalen) Sterile Solution (prescribing information). Therakos, Inc. 2. Knobler R, Arenberger P, Arun A, et al. European dermatology forum—updated guidelines on the use of extracorporeal photopheresis 2020—part 1. J Eur Acad Dermatol Venereol. 2020;34(12):2693-2716. 3. Kim EJ, Hess S, Richardson SK, et al. Immunopathogenesis and therapy of cutaneous T cell lymphoma. J Clin Invest. 2005;115(4):798-812. 4. Prince HM, Whittaker S, Hoppe RT. How I treat mycosis fungoides and Sézary syndrome. Blood. 2009;114(20):4337-4353. 5. Trautinger F, Eder J, Assaf C, et al. European Organisation for Research and Treatment of Cancer consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome. Eur J Cancer. 2017;77:57-74. 6. Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105(10):3768-3785. 7. Martinez XU, Di Raimondo C, Abdulla FR, et al. Leukaemic variants of cutaneous T-cell lymphoma: erythrodermic mycosis fungoides and Sézary syndrome. Best Pract Res Clin Haematol. 2019;32(3):239-252. 8. National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology. Primary Cutaneous Lymphoma. V1.2023. Accessed January 5, 2023. nccn.org 9. Diamandidou E, Cohen PR, Kurzrock R. Mycosis fungoides and Sezary syndrome. Blood. 1996;88(7):2385-2409. 10. Hwang ST, Janik JE, Jaffe ES, et al. Mycosis fungoides and Sézary syndrome. Lancet. 2008;371(9616):945-957. 11. Prip A, Moller KA, Nielsen DL, et al. The patient-healthcare professional relationship and communication in the oncology outpatient setting: a systematic review. Cancer Nurs. 2018;41:E11-E22. 12. Lucas AS, Ciccolini K. Nursing best practice referral algorithm for the early detection of mycosis fungoides. J Derm Nurs Assoc. 2016;8(2):109-120. 13. Sharma P, Wagner K, Wolchok JD, Allison JP. Novel cancer immunotherapy agents with survival benefit: recent successes and next steps. Nat Rev Cancer. 2011;11(11):805-812. 14. Bisaccia E, Gonzalez J, Palangio M, et al. Extracorporeal photochemotherapy alone or with adjuvant therapy in the treatment of cutaneous T-cell lymphoma: a 9-year retrospective study at a single institution. J Am Acad Dermatol. 2000;43(2 Pt 1):263-271. 15. THERAKOS® CELLEX® Photopheresis System: Operator’s Manual for Use With Software 5.4. 1470493_Rev06_EN-US. Mallinckrodt; 2020. 16. Hoen L, Pfeffer D, Schmidt JR, et al. Hydration status of geriatric patients is associated with changes in plasma proteome, especially in proteins involved in coagulation. Nutrients. 2023;15(17):3789. 17. Montoya GA, Bakuradze T, Eirich M, et al. Modulation of 3',5'-cyclic AMP homeostasis in human platelets by coffee and individual coffee constituents. Br J Nutr. 2014;112(9):1427-1437. 18. Sailer CO, Refardt J, Bissig S, et al. Effects of alcohol consumption on copeptin levels and sodium-water homeostasis. Am J Physiol Renal Physiol. 2020;318(3):F702-F709.
