title: Toxic epidermal necrolysis in a patient with primary myelofibrosis receiving thalidomide therapy.
abstract: Primary myelofibrosis (PMF) is a chronic myeloproliferative neoplasm characterized by progressive anemia, massive splenomegaly, leukoerythroblastosis, extramedullary hematopoiesis and in about 50% of cases the presence of JAK2V617F mutation. Curative therapy in PMF is currently possible only with allogeneic haematopoietic stem cell transplantation which is, unfortunately, associated with relatively high risks of mortality and morbidity which undermine its broad applications. Non-transplant treatment modalities are used for palliative purposes. Recently, anti-angiogenic drugs such as thalidomide have been used to treat these patients on the basis of the prominent bone marrow angiogenesis. Here, we report the case of a patient suffering from JAK2V617F-positive PMF with marked bone marrow neo-angiogenesis. The patient was treated with thalidomide but after 20 days developed life-threatening toxic epidermal necrolysis (TEN). To the best of our knowledge this is the first case of TEN in a patient with PMF under thalidomide therapy.
authors.affiliation: Chair of Hematology, Department of Internal Medicine and Public Health, University of L'Aquila, Via Vetoio snc, Coppito, 67100, L'Aquila, Italy.
authors.forename: Marianna
authors.initials: M
authors.lastname: Colagrande
authors.affiliation: Chair of Hematology, Department of Internal Medicine and Public Health, University of L'Aquila, Via Vetoio snc, Coppito, 67100, L'Aquila, Italy. mauro.diianni@cc.univaq.it.
authors.forename: Mauro
authors.initials: M
authors.lastname: Di Ianni
authors.affiliation: Department of Pathology, Ospedale San Salvatore, L'Aquila, Italy.
authors.forename: Gino
authors.initials: G
authors.lastname: Coletti
authors.affiliation: Department of Dermatology, University of L'Aquila, L'Aquila, Italy.
authors.forename: Ketty
authors.initials: K
authors.lastname: Peris
authors.affiliation: Department of Dermatology, University of L'Aquila, L'Aquila, Italy.
authors.forename: Maria Concetta
authors.initials: MC
authors.lastname: Fargnoli
authors.affiliation: Department of Clinical and Experimental Medicine, Hematology and Clinical Immunology Section, University of Perugia, Perugia, Italy.
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Moretti
authors.affiliation: Chair of Hematology, Department of Internal Medicine and Public Health, University of L'Aquila, Via Vetoio snc, Coppito, 67100, L'Aquila, Italy.
authors.forename: Mario
authors.initials: M
authors.lastname: Lapecorella
authors.affiliation: Chair of Hematology, Department of Internal Medicine and Public Health, University of L'Aquila, Via Vetoio snc, Coppito, 67100, L'Aquila, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Tabilio
mesh_terms: D000368:Aged; D006801:Humans; D008297:Male; D009389:Neovascularization, Pathologic / Q000188:drug therapy; D055728:Primary Myelofibrosis / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D013262:Stevens-Johnson Syndrome / Q000209:etiology* / Q000473:pathology; D013792:Thalidomide / Q000009:adverse effects*
chemical_list: D013792:Thalidomide