Identifying Potential Patients

Presenting with skin manifestations of CTCL unresponsive to other forms of treatment1

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

Mycosis fungoides 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.4

Sézary syndrome is 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.5,6

Typical treatment course for patients with skin manifestations of CTCL4,7

Initial treatment for patch/plaque disease

(skin-directed therapies)

CTCL diagnosis

(skin-directed therapies)

Refractory or progressive disease/symptom progression

(systemic therapies)

Refractory or progressive disease/symptom progression or aggressive growth

(systemic therapies, combination chemotherapies)

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Most patients with skin manifestations of CTCL experience slower progression of their underlying disease than patients with other forms of non-Hodgkin’s lymphoma (NHL) and should be treated accordingly.5,8

Given the chronic nature of CTCL, tolerability is an important consideration when choosing treatments.9

Real Patient Profiles

Kris, a patient treated with THERAKOS® Photopheresis

Actual photopheresis patient who has been compensated for sharing her story.

About Kris

Kris, a single mother, is working to fight the skin manifestations of CTCL.

Diagnostic history

An erythematous patch on Kris’s abdomen failed to respond to topical medications. Despite a nonspecific biopsy, her dermatologist suspected CTCL and prescribed a topical steroid. Lesions appeared intermittently for several years and then worsened to plaques with severe pruritus. A biopsy then confirmed the CTCL diagnosis.

Impact of CTCL skin manifestations

"Unbearable" pruritus reduced Kris’s quality of life. A topical cream calmed her skin symptoms during the day, allowing her to concentrate at work, but in the evenings, she scratched her skin until she bled.

Kris, a patient treated with THERAKOS® Photopheresis

Actual photopheresis patient who has been compensated for sharing his story.

About Lou

Lou, a retired Navy veteran, uses his courage to help him mentally fight the challenges of living with CTCL skin symptoms.

Diagnostic history

Onboard a US warship, 19-year-old Lou gave little thought to his "psoriasis" except to apply creams supplied by the ship’s infirmary. Twenty-five years later, with significant rash, scaling, and alopecia, the hospital biopsied him and diagnosed mycosis fungoides.

Impact of CTCL skin manifestations

Despite many treatments, Lou’s skin manifestations persisted. There were many times when Lou wanted to curl up and be a "hermit." For example, he was embarrassed by his skin flaking on friends’ furniture.

Start Patients With 2 Consecutive Days of THERAKOS® Photopheresis Every 4 Weeks1

Dosing can be adjusted based on patient response1

Initial/Standard Treatment Schedule

2 consecutive days
every 4 weeks

For a minimum of
7 cycles

If assessment during the fourth treatment cycle reveals a skin score that has worsened from baseline, the accelerated treatment schedule can be used.

Accelerated Treatment Schedule

2 consecutive days
every 2 weeks

For a maximum of
20 cycles

Patients should remain on continuous treatment for a minimum of 6 months before results can be fully assessed.

  • If a 25% improvement in the skin score is attained after 4 consecutive weeks on an accelerated treatment schedule, the standard treatment schedule may resume1

  • Patients who are maintained in the accelerated treatment schedule may receive a maximum of 20 cycles1

  • Treatment frequency exceeding labeling recommendations may result in anemia

  • There is no clinical evidence of additional benefit from treatment with UVADEX (methoxsalen) Sterile Solution beyond 6 months or on a different schedule1

Locating a treatment center for THERAKOS Photopheresis

There are more than 170 centers across the United States offering THERAKOS Photopheresis treatment, including most major US academic medical centers. The procedure is covered by most public and private insurers. Treatment centers are independent, third-party facilities not owned, operated, or controlled by Mallinckrodt Pharmaceuticals. This resource is provided for informational use only and may not be comprehensive.

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 increase a patient’s affinity for adherence.10 Here are some potential points that may help your patients receiving THERAKOS Photopheresis.

Photopheresis is not an immediate-response treatment

Clinical responses to immunotherapy may take longer to assess than with conventional agents because immunotherapy works by triggering an immune response.11*

An assessment is appropriate after the fourth treatment cycle, which is approximately 3 months after initiation. The approved course of treatment is a minimum of 7 cycles.1

*The exact mechanism of UVADEX is not known.

Set expectations that results may come, but it could take at least 3 to 6 months12

These minor lifestyle adjustments may help patients get ready

  • Hydrating prior to the procedure in anticipation of fluid shifts.13 Drinking enough water and juice while reducing alcohol and caffeine intake starting 2 days prior to a procedure is preferred

  • Avoiding high-fat foods the night before and on the day of the procedure.13 Low-fat, healthy meals are preferred

Set expectations that small changes may help prepare the patient for the procedure

References:

  1. UVADEX (methoxsalen) [prescribing information]. West Chester, PA: Therakos, Inc.; February 2018.

  2. Knobler R, Berlin G, Calzavara-Pinton P, et al. Guidelines on the use of extracorporeal photopheresis. J Eur Acad Dermatol Venereol. 2014;28(suppl 1):1-37.

  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. Trautinger F, Knobler R, Willemze R, et al. EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome. Eur J Cancer. 2006;42(8):1014-1030.

  5. Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. 2005;105(10):3768-3785.

  6. Konstantinow A, Balda B. Treatment of cutaneous T‐cell lymphoma with extracorporeal photochemotherapy. J Eur Acad Dermatol Venereol. 1997;9:111-117.

  7. National Comprehensive Cancer Network website. NCCN clinical practice guidelines in oncology: T-cell lymphomas. V5.2018. Available at: www.nccn.org/professionals/physician_gls/. Accessed October 4, 2019.

  8. Diamandidou E, Cohen PR, Kurzrock R. Mycosis fungoides and Sezary syndrome. Blood. 1996;88(7):2385-2409.

  9. Hwang ST, Janik JE, Jaffe ES, et al. Mycosis fungoides and Sézary syndrome. Lancet. 2008;371(9616):945-957.

  10. 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.

  11. 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.

  12. 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.

  13. THERAKOS® CELLEX® Photopheresis System: Operator’s Manual. Rev. 5.0-1460415. West Chester, PA: Therakos, Inc.; 2012.

INDICATIONS AND USAGE

UVADEX® (methoxsalen) Sterile Solution is indicated for extracorporeal administration with the THERAKOS® UVAR XTS® or THERAKOS® CELLEX® Photopheresis System in the palliative treatment of the skin manifestations of Cutaneous T-Cell Lymphoma (CTCL) that is unresponsive to other forms of treatment.

IMPORTANT SAFETY INFORMATION

CAUTION: READ THE THERAKOS UVAR XTS or THERAKOS CELLEX PHOTOPHERESIS SYSTEMS’ OPERATOR’S MANUAL PRIOR TO PRESCRIBING OR DISPENSING THIS MEDICATION.
UVADEX (methoxsalen) Sterile Solution should be used only by physicians who have special competence in the diagnosis and treatment of Cutaneous T-Cell Lymphoma and who have special training and experience in the THERAKOS UVAR XTS or THERAKOS CELLEX Photopheresis System. Please consult the appropriate Operator's Manual before using this product.

CONTRAINDICATIONS

UVADEX is contraindicated in patients exhibiting idiosyncratic or hypersensitivity reactions to methoxsalen, other psoralen compounds, or any of the excipients. Patients possessing a specific history of a light-sensitive disease state should not initiate methoxsalen therapy.

Diseases associated with photosensitivity include lupus erythematosus, porphyria cutanea tarda, erythropoietic protoporphyria, variegate porphyria, xeroderma pigmentosum, and albinism.

UVADEX is contraindicated in patients with aphakia because of the significantly increased risk of retinal damage due to the absence of lenses.

Patients should not receive UVADEX if they have any contraindications to the photopheresis procedure.

WARNINGS AND PRECAUTIONS

  • Patients who are receiving concomitant therapy (either topically or systemically) with known photosensitizing agents such as anthralin, coal tar or coal tar derivatives, griseofulvin, phenothiazines, nalidixic acid, halogenated salicylanilides (bacteriostatic soaps), sulfonamides, tetracyclines, thiazides, and certain organic staining dyes such as methylene blue, toluidine blue, rose bengal, and methyl orange may be at greater risk for photosensitivity reactions with UVADEX

  • Oral administration of methoxsalen followed by cutaneous UVA exposure (PUVA therapy) is carcinogenic. Methoxsalen also causes DNA damage, interstrand cross-links and errors in DNA repair

  • Methoxsalen may cause fetal harm when given to a pregnant woman. There are no adequate and well-controlled studies of methoxsalen in pregnant women. If UVADEX is used during pregnancy, or if the patient becomes pregnant while receiving UVADEX, the patient should be apprised of the potential hazard to the fetus. Women of childbearing potential should be advised to avoid becoming pregnant. It is not known whether this drug is excreted in human milk

  • After methoxsalen administration, exposure to sunlight and/or ultraviolet radiation may result in "premature aging" of the skin

  • Since oral psoralens may increase the risk of skin cancers, monitor closely those patients who exhibit multiple basal cell carcinomas or who have a history of basal cell carcinomas

  • Serious burns from either UVA or sunlight (even through window glass) can result if the recommended dosage of methoxsalen is exceeded or precautions are not followed

  • Patients should be advised to avoid all exposure to sunlight during the 24 hours following photopheresis treatment

  • Exposure to large doses of UVA light causes cataracts in animals. Oral methoxsalen exacerbates this toxicity. Serum methoxsalen concentrations are substantially lower after extracorporeal UVADEX treatment than after oral methoxsalen treatment. Nevertheless, if the lens is exposed to UVA light while methoxsalen is present, photoactivation of the drug may cause adducts to bind to biomolecules within the lens

  • Instruct patients emphatically to wear UVA-absorbing, wrap-around sunglasses for 24 hours after UVADEX treatment

  • Safety in children has not been established

  • Thromboembolic events, such as pulmonary embolism and deep vein thrombosis, have been reported with UVADEX administration through photopheresis systems for treatment of patients with graft-versus-host disease, a disease for which UVADEX is not approved.

ADVERSE REACTIONS

Side effects of photopheresis (UVADEX used with the THERAKOS Photopheresis System) were primarily related to hypotension secondary to changes in extracorporeal volume (>1%)
For the THERAKOS® UVAR XTS®/CELLEX® Photopheresis Procedure:

INDICATIONS

The THERAKOS UVAR XTS Photopheresis System/THERAKOS CELLEX Photopheresis System is indicated for use in the ultraviolet-A (UVA) irradiation, in the presence of the photoactive drug 8-methoxypsoralen (8-MOP®), of extracorporeally circulating leukocyte-enriched blood, in the palliative treatment of the skin manifestations of Cutaneous T-Cell Lymphoma (CTCL), in persons who have not been responsive to other forms of treatment.

IMPORTANT SAFETY INFORMATION

CONTRAINDICATIONS

The THERAKOS UVAR XTS or THERAKOS CELLEX Photopheresis Systems are not designated, sold, or intended for use except as indicated.

Certain underlying medical conditions contraindicate THERAKOS Photopheresis, including patients:

  • who cannot tolerate extracorporeal volume loss during the leukocyte-enrichment phase

  • exhibiting idiosyncratic or hypersensitivity reactions to 8-methoxypsoralen/psoralen compounds

  • with coagulation disorders

  • who have had previous splenectomy

WARNINGS AND PRECAUTIONS

  • THERAKOS Photopheresis treatments should always be performed in locations where standard medical emergency equipment is available. Volume replacement fluids and/or volume expanders should be readily available throughout the procedure

  • Patients who may not be able to tolerate the fluid changes associated with extracorporeal photopheresis should be monitored carefully

  • Procedures, such as renal dialysis, which might cause significant fluid changes (and expose the patient to additional anticoagulation) should not be performed on the same day as extracorporeal photopheresis

  • Individual patients may require a heparin dosage that varies from the recommended dose to prevent post-treatment bleeding or clotting during a treatment

ADVERSE REACTIONS

  • Hypotension may occur during any treatment involving extracorporeal circulation. Closely monitor the patient during the entire treatment for hypotension

  • Transient pyretic reactions, 37.7-38.9°C (100-102°F), have been observed in some patients within six to eight hours of reinfusion of the photoactivated leukocyte-enriched blood. A temporary increase in erythroderma may accompany the pyretic reaction

  • Treatment frequency exceeding labeling recommendations may result in anemia

  • Venous access carries a small risk of infection and pain

Please see accompanying Full Prescribing Information, including the BOXED WARNING for UVADEX, and see the appropriate THERAKOS Photopheresis System Operator’s Manual.
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