Suggested Guidelines for the Treatment of Mycosis Fungoides in Countries with Limited Resources

Figure 3

Algorithm for treatment of classic MF/SS. The treatment is presented in a stepwise pattern where patients shift to the next line of therapy in case of the absence of complete response (CR) after 1 year of the current line of therapy, no response after 3–6 months, or the occurrence of progressive disease (PD). For early MF, phototherapy is given where PUVA is more recommended than NB-UVB for our patients with dark skin phenotype (level 2). Potent steroids (level 3) and moisturizers (level 5) are additional basic treatment. Addition of either RAR agonist (level 2) or methotrexate (level 5) is recommended as a second line. Moreover, methotrexate can be combined with acitretin (level 4). TSEB is considered a third line option (level 2). Topical tazarotene (level 3) or gentian violet (level 5) can be added to resistant patches; and localized radiotherapy (level 4) or gentian violet can be added to resistant plaques. Patients with stages (IB-IIA) who show CR should enter a maintenance and follow-up regimen (level 2). For stage IIB with limited disease (up to 3 nodules), localized radiotherapy can be added to SDT (level 4). For stage IIB with multiple nodules, stage III and SS, PUVA plus RAR agonists (level 2) or methotrexate (level 5) or combined methotrexate and acitretin is recommended as a first line (level 4), TSEB is considered a second line option (level 2). Monochemotherapy with gemcitabine or liposomal doxorubicin can be given instead (level 4). Polychemotherapy and allogenic HCT are regarded as a final option (level 3). For stage IV, monochemotherapy is the first line (level 4), followed by polychemotherapy and allogenic stem cell transplantation (level 3). Acitretin can be combined with methotrexate. TSEB is preferable than monochemotherapy if feasible to the patient. Phototesting and slow dose escalation are mandatory in case of erythroderma in stage III and IV. CR: complete response, PD: progressive disease, FU: follow-up, TSEB: total skin electron beam, GV: gentian violet, RT: radiotherapy, RAR: retinoic acid receptor, EBRT: external beam radiotherapy, HCT: hematopoietic cell transplantation.