Mycosis fungoides' therapies make Tx 'rewarding'.

SAN ANTONIO -- The treatment of most patients who have mycosis fungoides is well within the purview of dermatologists.

"It's very rewarding caring for these patients, because they respond to so many different therapies," Dr. L. Frank Glass said at the annual meeting of the American Academy of Dermatology.

In fact, most patients do very well and don't die from this disease, which is the most common of the cutaneous T-cell lymphomas. Its treatment resembles that of a chronic disease, such as psoriasis, more than that of a terminal illness, said Dr. Glass of the University of South Florida, Tampa.

From topical steroids and retinoids to alkylating agents and phototherapy, the range of treatments for cutaneous T-cell lymphomas is expansive and provides options for successfully treating the many variants of the disease. Furthermore, these are treatments with which dermatologists, compared with other specialists, have the most extensive experience.

"We're used to using retinoids orally," he said, noting that even imiquimod--another drug with which dermatologists have extensive experience--is considered an off-label option for the treatment of mycosis fungoides.

Dermatologists will mainly see patients with disease up to the limited patch-plaque stage. Disease progression in these patients tends to be limited, and some patients with very early-stage disease have been shown to have the same survival as the general population.

At least one study has shown that those patients who receive conservative sequential therapy do as well as those patients who receive aggressive chemotherapy and radiation treatment, Dr. Glass explained.

Even with patients who have more advanced tumor-stage disease that requires multispecialty care, dermatologists can continue to play a role in treatment, thanks to the following modalities now available:

* Topical corticosteroids. Class I corticosteroids tend to work best for mycosis fungoides patients, but should be avoided in those with atrophic disease variants. When they are appropriate, corticosteroids tend to work well for itching and redness. Corticosteroids are particularly useful as adjuvants with other therapies.

* Phototherapy. PUVA is best, but narrow-band phototherapy offers more convenience and also has good results. The choice depends on the stage and type of lesion, as well as patient risk factors, Dr. Glass said.

Although response rates are similar with PUVA and narrow-band light, there are some questions about the durability of response with narrow-band light and whether thicker lesions respond as well to this form of phototherapy. In those with higher melanoma risk, however, narrowband light may be safer.

Phototherapy is great as a maintenance therapy, but in those with progressive disease, relapse rates increase and combination therapy with a systemic agent may be necessary.

* Extracorporeal photophoresis. This treatment is quite effective, particularly in patients with erythrodermic mycosis fungoides and Sezary syndrome. It is the treatment of choice for the former, and is "the beginning of treatment" for the latter, he said.

* Bexarotene. Topical bexarotene is promising, with a similar mechanism of action to other retinoids, but at nearly $2,000 per tube it is too expensive to be considered a viable option at this time, Dr. Glass noted.

Oral bexarotene is more accessible and works well in combination with other therapies, such as PUVA.

Using oral bexarotene in a combination allows the dosage of both agents to be reduced, thus maintaining good response but reducing the risk of adverse effects, such as hypertriglyceridemia and hypothyroidism.

However, Dr. Glass noted that when he uses bexarotene in combination with another treatment for sustained therapy, he brings in an internist to ensure that the patient doesn't have an increased cardiac risk or a metabolic problem "down the road."

Nonetheless, combination therapy is "pretty effective and can certainly be initiated" by dermatologists with fewer side effects, he said.

BY SHARON WORCESTER

Southeast Bureau