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MF/SS: Systemic Therapy

CME

New Systemic Therapies for Mycosis Fungoides and Sézary Syndrome

Physicians: Maximum of 1.00 AMA PRA Category 1 Credit™

Released: March 05, 2020

Expiration: March 04, 2021

CME/CE Information

Activity

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In this module, Francine Foss, MD, provides an overview of the diagnosis and treatment of patients with mycosis fungoides (MF) or Sézary syndrome (SS), with a focus on the most recently approved systemic therapies.

The key points discussed in this module are illustrated with thumbnails from the accompanying downloadable PowerPoint slideset; they can be found here or downloaded by clicking on any of the slide thumbnails in the module alongside the expert commentary.

Clinical Care Options plans to measure the educational impact of this activity. A few questions will be asked twice: once before the discussion that informs the best choice and then again after that specific discussion. Your responses will be aggregated for analysis, and your specific responses will not be shared.

Before continuing with this educational activity, please take a moment to answer the following questions.

If you are a practicing clinician, how many patients with MF or SS do you see in an average year?

DT is a 67-year-old male who presented with extensive infiltrated plaques. He was initially diagnosed with psoriasis and was treated with topical steroids and UVB, with some response. Lesions progressed over 2 years, and he developed a rapid onset of cutaneous tumors, some of which were ulcerating.


Biopsy showed tumor-stage mycosis fungoides with epidermotropism and deep dermal infiltration. Immunophenotyping of the tumor tissue showed that the malignant cells were CD4+CD7-CD26-, and 20% of the tumor cells stained for CD30. Flow cytometry of peripheral blood showed no Sézary cells. T-cell gene rearrangement in the skin showed a dominant clone. Lactate dehydrogenase (LDH) was elevated at 430 U/L. PET scan showed uptake with a standard uptake value (SUV) of 5-7 in axillary and inguinal nodes, which were enlarged, and uptake in skin lesions. Radiation was applied to ulcerating lesions.

In your current practice, which of the following treatment options would you recommend for this patient?

BG is an 85-year-old woman who presented with a skin rash and pruritus for 4 months. On examination, she had erythroderma involving nearly 100% of her body surface area, mild exfoliation of skin over the trunk and extremities, and fissures on the palms and soles. She reported feeling cold all the time but had no fevers. She used topical steroids with no relief; itching kept her up all night.


Skin biopsy showed epidermotropism with extensive deep dermal infiltrate suggestive of mycosis fungoides. Laboratory data: white blood cell 4.9 x 109/L and flow cytometry showed that 18% of lymphocytes were CD4+CD2+CD5+CD7, consistent with Sézary cells. T-cell receptor rearrangements were positive in the blood. CT scan showed axillary and inguinal adenopathy measuring 1.5-2.0 cm.


The patient was treated with ECP with minimal response then bexarotene and interferon alfa were added. She had a modest response but continued to have erythema and pruritus with rising LDH.

In your current practice, which of the following treatment options would you recommend for this patient?

Which of the following is the target of mogamulizumab?
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