title: Transformation of Sézary syndrome into CD30+ anaplastic large T-cell lymphoma after alemtuzumab therapy with evidence of clonal unity.
abstract: Alemtuzumab is a humanized mouse antibody targeting the CD52 cell surface, which has been effective in patients with advanced stage mycosis fungoides (MF) including erythrodermic MF and Sézary syndrome. There are a few descriptions of large cell transformation after its administration. A young patient with an acute onset of Sézary syndrome treated initially unsuccessfully with fludarabine and cyclophosphamide and later on successfully with alemtuzumab has been described. Three weeks after the beginning of therapy, however, she developed transformed T-cell lymphoma indistinguishable from CD30 anaplastic large-cell lymphoma. After bone marrow transplantation, the transformed CD30 cutaneous T-cell lymphoma recurred as a transformed CD30 plaque MF. All 3 types of lesions showed the same T-cell receptor clonal gene rearrangement, which supports the notion that Sézary syndrome, CD30 anaplastic large-cell lymphoma, and MF are interrelated.
authors.affiliation: *Department of Dermatology, Rambam Health Care Campus and the Bruce Rappaport Faculty of Medicine, Technion, Institute of Technology, Haifa, Israel, and †Division of Hematology, Rambam Health Care Campus and the Bruce Rappaport Faculty of Medicine, Technion, Institute of Technology, Haifa, Israel.
authors.forename: Mariela Judith
authors.initials: MJ
authors.lastname: Nevet
authors.forename: Tsila
authors.initials: T
authors.lastname: Zuckerman
authors.forename: Dvora
authors.initials: D
authors.lastname: Sahar
authors.forename: Reuven
authors.initials: R
authors.lastname: Bergman
mesh_terms: D000328:Adult; D000074323:Alemtuzumab; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D014408:Biomarkers, Tumor / Q000032:analysis*; D001706:Biopsy; D016026:Bone Marrow Transplantation; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte; D019672:Genes, T-Cell Receptor; D006801:Humans; D007150:Immunohistochemistry; D017730:Ki-1 Antigen / Q000032:analysis*; D017728:Lymphoma, Large-Cell, Anaplastic / Q000235:genetics / Q000276:immunology* / Q000473:pathology / Q000601:surgery; D009182:Mycosis Fungoides / Q000235:genetics / Q000276:immunology* / Q000473:pathology / Q000628:therapy; D012751:Sezary Syndrome / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology / Q000473:pathology; D012878:Skin Neoplasms / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology / Q000473:pathology; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D000970:Antineoplastic Agents; D014408:Biomarkers, Tumor; D017730:Ki-1 Antigen; D000074323:Alemtuzumab