Toxic epidermal necrolysis in a patient with primary myelofibrosis receiving thalidomide therapy.
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.
Colagrande
Marianna
M
Chair of Hematology, Department of Internal Medicine and Public Health, University of L'Aquila, Via Vetoio snc, Coppito, 67100, L'Aquila, Italy.
Di Ianni
Mauro
M
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.
Coletti
Gino
G
Department of Pathology, Ospedale San Salvatore, L'Aquila, Italy.
Peris
Ketty
K
Department of Dermatology, University of L'Aquila, L'Aquila, Italy.
Fargnoli
Maria Concetta
MC
Department of Dermatology, University of L'Aquila, L'Aquila, Italy.
Moretti
Lorenzo
L
Department of Clinical and Experimental Medicine, Hematology and Clinical Immunology Section, University of Perugia, Perugia, Italy.
Lapecorella
Mario
M
Chair of Hematology, Department of Internal Medicine and Public Health, University of L'Aquila, Via Vetoio snc, Coppito, 67100, L'Aquila, Italy.
Tabilio
Antonio
A
Chair of Hematology, Department of Internal Medicine and Public Health, University of L'Aquila, Via Vetoio snc, Coppito, 67100, L'Aquila, Italy.
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*
D013792:Thalidomide