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Leukemik Mycosis Fungoides with Follicular Mucinosis : Case Report

Case of a 38-year-old female patient with the leukemic form of mycosis fungoides (MF) stage IV A (B), the course of the disease and treatment are presented. The diagnosis of MF (stage IB) was made in 2002. In the next year, the patient received PUVA therapy, which resulted in disease remission ; however, in September 2005, deterioration of the skin state occurred with rapid appearance of numerous infiltrates and tumors on the skin, axillary and inguinal lymph node enlargement, and leukemic blood changes, with the development of leonine facies and pronounced folliculotropic manifestations (follicular mucinosis). Chemotherapy was introduced (Leukeran, methotrexate, prednisone). Upon short-term remission, relapse of the disease required interferon treatment. Photopheresis (24 cycles) was performed from May to December 2006, along with 11 cycles of Gemzar chemotherapy. The patient was occasionally administered antibiotic therapy for infectious complications. The patient was closely monitored, with an array of supportive measures. In spite of intensive therapy, skin lesions progressed, with persistent leukocytosis, lymphocytosis and lymph node involvement. At the beginning of 2007, the patient received two cycles of combined chemotherapy (Setronon, Fludara, Dexasone), followed by three cycles of Caelyx. As the skin lesions continued progressing with aggravation of the patient's general condition, Targretin was introduced in therapy at a dose of 75 mg, however, with no success. As tumor enlargement persisted in spite of manifold therapy, large tumors on the face and trunk were treated with superficial soft x-ray radiotherapy, along with the administration of methotrexate, 30 mg once a week. The patient's general condition was additionally burdened by frequent infections, yet longer remissions were achieved. Currently, the patient is receiving vorinostat therapy. The management of aggressive skin lymphomas is very complex and requires sophisticated equipment, frequent evaluation of therapeutic results, and readiness for intervention in critical situations.

Leukemic mycosis fungoides; follicular mucinosis; chemotherapy

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