Receiving a diagnosis of mycosis fungoides (MF) can be very sobering for the unfortunate patient. However, although there is no cure for this rare type of lymphoma, several different therapies are currently being used that can lead to long-term disease remission, significantly increasing the quality of life of patients. Physicians not only need to arrive at the correct diagnosis of MF using a constellation of different diagnostic parameters and tests, but also convey the “right” message to the patient concerning their treatment, management, as well as prognosis, says Jo-Ann M. Latkowski M.D., a dermatologist with the NYU Langone Medical Center.
“As of yet, there is no cure for mycosis fungoides, but a lot can be done to achieve long-term disease-free intervals. As such, patients who are diagnosed with MF, particularly those patients with early stage disease could be offered cautious optimism by their doctor. They need to be given a careful message concerning the stage of their disease and given hope in terms of achieving disease remission,” Dr. Latkowski says.
As the most common type of cutaneous T-cell lymphoma (CTCL), MF may present with a number of signs and symptoms including thin wrinkled erythematous patches on the skin, raised plaques, tumor or nodular skin changes, and/or erythroderma. Within these pathological lesions, skin sample histopathology will typically find an abnormal accumulation of T-cells that multiply excessively and uncontrolled, and it is the interaction of these malignant lymphocytes with other cells in the skin that cause the cutaneous symptoms seen in MF patients.
In addition to a thorough skin exam that can already invoke suspicion of MF, clinicians should perform a number of lab exams to help them arrive at the correct diagnosis and staging.
One cornerstone examination is the TCR rearrangement study, which when found positive could indicate MF disease. In early-stage MF patients, however, Dr. Latkowski says that the PCR (polymerase chain reaction) exam performed here may be inconclusive due to several factors including the presence of fewer malignant T cells, tissue sampling error, as well as the presence of reactive T cells admixed with neoplastic T cells, the latter not occurring infrequently in patients with longer standing benign dermatoses. Clonal T cell populations have been documented in the literature in benign dermatologic diseases and conditions as well, including psoriasis, lichen planus, lichen sclerosis et atrophicus, pityriasis lichenoides et varioliformis acuta, pityriasis lichenoides chronica, and contact dermatitis.
“It must be made very clear that clonality does not equal malignancy and as such, a positive TCR gene rearrangement study does not confirm the diagnosis of MF. The results of PCR exams cannot be used as a sole criterion for establishing a diagnosis of MF. The results must be appropriately correlated with clinical morphology and histopathological findings in order to better hone in on an accurate diagnosis of MF,” Dr. Latkowski says.
Several different skin-directed therapies have been tried to address and quell the signs and symptoms of MF including high potency corticosteroids, topical bexarotene, topical nitrogen mustard, phototherapy, localized radiation and electron beam radiation.
Choosing the appropriate therapy depends on a number of different factors including the patient’s age, disease stage, existing co-morbidities as well as the patient’s personal goals in terms of aiming for a remission or palliative therapy. It remains unclear as to which therapy may work best and according to Dr. Latkowski the choice of therapy should be individualized in each MF patient.
Regardless of the durable disease remissions that can be achieved with the currently applied therapies, patients must always be well prepared by their doctor in terms of potential recurrences.
“I think we need to really set our expectations for the patients that a recurrence can happen, and if it is early stage disease (i.e. IA-IB), it shouldn’t trigger anxiety in our patients. They need to understand that what they have is an indolent disease and even if it comes back, it doesn’t mean that it is progressing to a more advanced stage,” Dr. Latkowski says.
Physicians who see these patients have to learn how to better communicate and speak with them and transfer the right message. They should give their patients a clear roadmap in respect to the severity of their disease stage and current presenting symptoms, and the possibility to achieve disease remission with appropriate therapy.
“You can’t rely on one factor to make the diagnosis. It’s a clinical pathological correlation to make the diagnosis, and once the patient has the diagnosis, especially in early stage disease, the majority of patients follow an indolent disease course. Although MF it is not curable, it is controllable and the ultimate goal would be to achieve long-term remission,” Dr. Latkowski says.
Disclosures: Dr. Latkowski reports no relevant disclosures.