Haema 2010; 1(1): 72-84
by Evaggelia Papadavid1, Marina Siakantaris2 and Christina Antoniou3
1Ass. Professor of Dermatology, 2nd Department of Dermatology, National and Kapodistrian University of Athens,
2Ass. Professor of Internal Medicine, 1st Department of Internal Medicine, National and Kapodistrian University of Athens,
3Professor of Dermatology, 1st Department of Dermatology, National and Kapodistrian University of Athens
Primary cutaneous T cell lymphomas (CTCL) are rare lymphoproliferative disorders accounting for the 80% of primary cutaneous lymphomas (PCL). The skin is initially affected and other organs are usually affected only in advanced stages. Mycosis Fungoides (MF) is the commonest PCL (60%) with indolent course, followed by lymphomatoid papulosis, primary CD30+ anaplastic cutaneous lymphoma and subcutaneous T cell lymphoma panniculitis like. Patients’ stage at diagnosis is a very important factor for treatment recommendation, disease prognosis and overall survival. An overall survival similar to general population has been shown in early stage MF and therapeutic recommendations are for skin directed therapies. CTCL is responsive to current treatment modalities but generally an incurable disease. Recurrences are common and it is unclear whether therapeutic modalities affect overall disease survival. Patients are candidates for systemic therapy if they present with advanced stage or have progressed under skin directed treatment. The immunomulatory agents such as interferon-α or rexinoids should be initially tried, which produce good overall responses. Fusion proteins as denileukin diftitox or methotrexate can be added to the treatment regimen. Patients who do not respond to biologic agents, should be treated with chemotherapy. Single or combined chemotherapeutic agents result in rapid responses and high response rates but are highly immunosuppressive and don’t offer durable responses. There are a number of alternative approaches especially for SS as the anti-CD52 monoclonal antibody which demonstrates high responses clearing malignant lymphocytes from the blood, and extracorporeal photopheresis alone or combined with low-dose interferon or bexarotene for erythrodermic MF and SS. Radiotherapy either in the form of localized irradiation or as total skin electron beam therapy (TSEBT), has proved an excellent therapeutic tool for resistant cutaneous lesions. Novel therapies with promising results are available or in development. Histone deacetylase inhibitors (HDAC) target tumor suppressor genes providing and antitumor effect. Finally, the role of allogeneic hematopoietic stem cell transplantation needs further evaluation as it may be a successful treatment for patients resistant to currently available therapeutic modalities.