title: Sezary syndrome presenting with leonine facies and treated with low-dose subcutaneous alemtuzumab.
abstract: Cutaneous T-cell lymphomas (CTCL) comprise a group of diseases characterized by the accumulation of malignant T cells within the skin. Sezary syndrome represents an aggressive form of CTCL, in which the skin is diffusely affected and the peripheral blood is involved. It is characterized by the triad of generalized erythroderma, lymphadenopathy, and neoplastic T cells (Sezary cells) in the skin, lymph nodes, and peripheral blood. Leonine facies is rare and corresponds to the morphologic manifestation of diffuse dermal infiltration of the face. It can occur in cutaneous T-cell lymphomas that progress during years without therapy. We present the case of a 54-year-old man with Sezary syndrome presenting with leonine facies, unresponsive to conventional therapies; he exhibited a promising response to subcutaneous low-dose alemtuzumab.
authors.affiliation: Hospital de Santo António, Centro Hospitalar do Porto, Porto, Portugal.
authors.forename: Ana
authors.initials: A
authors.lastname: Oliveira
authors.forename: Inês
authors.initials: I
authors.lastname: Lobo
authors.forename: Rosário
authors.initials: R
authors.lastname: Alves
authors.forename: Margarida
authors.initials: M
authors.lastname: Lima
authors.forename: Manuela
authors.initials: M
authors.lastname: Selores
mesh_terms: D000074323:Alemtuzumab; D061067:Antibodies, Monoclonal, Humanized / Q000008:administration & dosage / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use; D001706:Biopsy; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D019008:Drug Resistance, Neoplasm; D005148:Facial Dermatoses / Q000209:etiology; D019066:Facies; D006801:Humans; D016130:Immunophenotyping; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000008:administration & dosage; D011537:Pruritus / Q000209:etiology; D012074:Remission Induction; D012751:Sezary Syndrome / Q000175:diagnosis* / Q000188:drug therapy; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000188:drug therapy; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D000074323:Alemtuzumab; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone