Clinical characteristics and long-term outcome of patients with generalized patch and/or plaque (T2) mycosis fungoides

Objectives: To study the long-term results of treatment of patients with ge neralized patch and/or plaque mycosis fungoides and to identify clinical ch aracteristics predictive of survival and response to treatment. Design: A single-center, 35.5-year retrospective cohort analysis. Setting: Private referral medical center. Patients: One hundred seventy-six patients with generalized patch and/or pl aque (T2) mycosis fungoides. Main Outcome Measures: Long-term actuarial survival and freedom-from-relaps e results as calculated by the Kaplan-Meier method. Results: The long-term (35.5-year) survival of patients with T2 mycosis fun goides is worse than the expected survival of a race-, age-, and sex-matche d control population (P < .001). The median survival of the T2 group is 11. 7 years. Patients younger than 58 years (median age) at presentation have a more favorable overall and disease-specific survival. than the patients wh o are 58 years or older (P < .001 vs P < .025). Patient sex or race had no significant effect on overall survival. Patients who presented with palpabl e clinically significant lymph nodes (stage IIA) had long-term survival res ults similar to those without lymphadenopathy (stage IB), despite improved freedom-from-relapse outcome for patients with stage IB. Twenty-four percen t of patients who progressed to more advanced disease had a lower complete response rate to initial therapy than did other patients (21% vs 65%) (P < .001). Patients who received total skin electron beam therapy had a better complete response rate than patients treated with topical mechlorethamine h ydrochloride alone; the relapse-free results were superior in patients with a total dose of 30 Gy or higher and in patients who received topical mechl orethamine as adjuvant therapy following total skin electron beam therapy. Despite differences in freedom-from-relapse results among different treatme nt groups, long-term overall or disease-specific survivals were not signifi cantly different. Conclusions: A significant proportion (24%) of patients with generalized pa tch and/or plaque (T2) mycosis fungoides experience disease progression to a more advanced clinical stage, and nearly 20% eventually die of the diseas e. Younger patients have a more favorable disease-specific long-term outcom e than patients who are older. Presence of lymphadenopathy (stage IIA) at d iagnosis does not predict worse long-term survival outcome. Clinical featur es predictive of disease progression include initial lymphadenopathy (stage IIA) and lack of complete response to initial treatment. Despite superior complete response rate to a 30-Gy or higher dose of total skin electron bea m therapy, topical mechlorethamine proves to be a cost-effective initial tr eatment for patients with T2 disease. The concept of an adjuvant therapy af ter irradiation is appealing, although it may not lead to improved long-ter m survival.