The long-term complete remission results of a recent case study using Extracorporeal photopheresis (ECP) in early-stage mycosis fungoides (MF) suggests a wider utility for the treatment modality, offering a beacon of hope for this difficult-to-treat patient population.
ECP is largely considered to be a useful treatment modality for advanced-stage MF; however, little is known of its therapeutic effect in early-stage (IA-IIA) disease.
In the past, ECP has commonly been used for the treatment of cutaneous T-cell lymphoma (CTCL), with current clinical practice largely reserving the ambulatory therapy for patients with late-stage MF or Sézary syndrome (the leukemic form of CTCL), or for those patients who are refractory to other treatments.
ECP has not been widely used in early-stage disease, in part because it is thought to require MF involvement of the blood to be effective. As such, the treatment has potentially been underused in patients with early-stage disease, as skin-directed therapies including topical corticosteroids and topical nitrogen mustard, among others, are typically prescribed as first-line therapies.
“There are several different treatment approaches one can choose from when considering therapy for MF,” says Daniel J. Lewis, M.D., Candidate and Research Assistant, Department of Dermatology, The University of Texas MD Anderson Cancer Center, Houston, Texas, and head author of the study.
“Considering the high efficacy and safety of ECP, we believe the therapy is underused in this patient population. Particularly in contrast to the currently available treatments, ECP is one treatment modality that can potentially circumvent the side effects associated with other used therapies, making it a potential favorite among patients and their physicians.”
Early-stage MF therapy case study
In the case study1, researchers employed ECP therapy in a 48-year-old Caucasian male with diagnosed early-stage patch MF located on his right lateral thigh and buttock, involving 7% of his total body surface area. Over the course of five years, the patient underwent 36 cycles of two treatments each and achieved complete remission of his patch MF disease. At the final follow-up visit, a lymphoma panel and flow cytometry of the blood showed changes consistent with a decreased Th2 cytokine profile and increased Th1 profile.
Mycosis fungoides involves an immune system dysregulation whereby there is a cytokine imbalance; specifically, instead of having a balance between the Th1 and Th2 cytokine profiles, MF has a Th2 cytokine predominance. According to the researchers, ECP is capable of correcting this imbalance and results in an increase of Th1 cytokines.
Though the standard therapies for early MF including topical corticosteroids, topical nitrogen mustard, and light therapy may be effective, each treatment has disadvantages in the long-term (i.e. skin atrophy, allergic contact dermatitis, increased risk of skin cancers, respectively) and are therefore, in part, considered suboptimal treatment solutions.
“The commonly used therapies, such as topical steroids and phototherapy, are not able to correct the cytokine imbalance occurring in MF because they are skin-directed treatments. In stark contrast, though, ECP affects the blood and can effectively correct the cytokine imbalance without any of the side effects associated with the standard therapies,” Dr. Lewis says.
Although it has been shown to circumvent the side effects associated with standard therapies, ECP can be somewhat time-consuming. Instead of patients simply applying steroids at home, they have to come in for ECP treatment perhaps once a month for a three-hour therapy. Nevertheless, the evidence indicates that patients may be better off in the long-term considering the possibility of complete remission devoid of adverse events.
“We’ve had a lot of these patients do extremely well with ECP in early-stage MF. Patients do not mind coming in once a month for their therapy, particularly when offered the option of other available therapies,” Dr. Lewis says.
Approximately 10% of patients will progress from early-stage MF to more progressive disease including Sézary syndrome. According to the researchers, it is thought that ECP may prevent progression of MF to advanced disease stages.
“Instead of prescribing topical steroids which might treat the cutaneous symptoms effectively but might allow for the disease to progress, perhaps we should be using ECP more often,” Dr. Lewis suggests. “The therapy may be associated with decreased disease progression because we are not only treating the skin but also the blood, resulting in correction of the immune system dysregulation. By using ECP, perhaps we are preventing progression of early-stage disease to advanced-stage MF. In addition to the skin response, ECP also works as a systemic immunomodulatory therapy and appears to have a systemic immunologic response.”
Disclosure: Dr. Lewis reports no relevant disclosures.
1 Lewis DJ, Duvic M; Dermatol Ther. 2017 Mar 24. doi: 10.1111/dth.12485. [Epub ahead of print]. Extracorporeal photopheresis for the treatment of early-stage mycosis fungoides.