Author disclosures Alejandro Ariel Gru is a paid consultant for Seattle Genetics and has an ongoing relationship with Innate Pharma. John Zic is a site principal investigator for clinical trials for products from Elora and Galderma. The other authors report no actual or potential conflicts of interest with regard to this article.
Disclaimer The opinions expressed herein are those of the authors and do not necessarily reflect those of Federal Practitioner, Frontline Medical Communications Inc., the US Government, or any of its agencies. The manufacturers did not provide equipment or other forms of material support.
Lauren Pinter-Brown. I use radiation therapy sparingly and primarily for patients who either have only 1 tumor and the rest of their disease is patch and plaque or for patients who have very large tumors that are either cosmetically unacceptable or creating infection or pain. I treat people with systemic therapies primarily to prevent the formation of tumors.
John Zic. There probably is a role for total skin electron beam radiotherapy in patients who have failed multiple other skin-directed therapies and are progressing and then perhaps a role for more advanced patients who have multiple tumors where you’re trying to get some control of the disease. Are there any other situations where you might consider total skin electron beam?
Marianne Tawa. Yes, those are 2 scenarios. A third scenario would be in patients preparing for stem cell transplant. We typically do a modified 12 Gy regimen of total skin electron beam for palliation and up to 24 Gy regimen for patients who are in earnest preparing for a stem cell transplant.
John Zic. Systemic therapies also treat this disease. There are 2 oral agents. One is bexarotene capsules, a retinoid that binds to the RXR receptor and has a multitude of effects on different organ systems. It is probably the best tolerated oral agent we have. The other FDA-approved agent is vorinostat, a histone deacetylase inhibitor, but it has more gastrointestinal AEs than does bexarotene. Bexarotene has AEs as well, including hypertriglyceridemia and central hypothyroidism, which can throw a curveball to unsuspecting primary care physicians who might check thyroid function studies in these patients.
We certainly need to know about those AEs. There are many patients who have tumor-stage disease that can have radiotherapy to several tumors, then go on a drug like bexarotene capsules and may be able to maintain the remission for years. In my experience, it’s a drug that patients usually stay on. They can be weaned to a very low dose, but I’ve had several patients who come off of bexarotene only to suffer relapses.
Lauren, what are some of the things that you think about when you declare someone as having failed bexarotene or vorinostat and you’re thinking about IV therapies?
Lauren Pinter-Brown. Patient comorbidities and the particular compartment of their body that is involved are important factors. Do they have blood involvement, or not? Do they have nodal involvement, or not? Another concern is both acute and chronic toxicities that need to be discussed with the patient to determine an acceptable QOL. Finally, the schedule that you’re giving the drug. Some people may not be able to come in frequently. There are a lot of variables that go into making an individual decision at a particular time for a specific patient who will be using parenteral therapies.
John Zic. If we have a patient with advanced MF, tumors, and perhaps lymph node involvement, what are some of the systemic options that you would consider?
Lauren Pinter-Brown. With nodal involvement, an attractive option is something like IV romidepsin because we know that it treats peripheral T-cell lymphomas, which are aggressive nodal T-cell lymphomas. It’s FDA approved and also treats CTCL. Another is brentuximab vedotin if there is significant CD30 expression. It also is FDA approved for CTCL and has a long track record of treating certain peripheral T-cell lymphomas like anaplastic large cell.