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Toxic epidermal necrolysis in a patient with primary myelofibrosis receiving thalidomide therapy

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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.

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Acknowledgments

We would like to thank Dr. Geraldine Anne Boyd for her help. This work was supported by “Associazione Italiana Leucemie, Linfomi e Mielomi”, L’Aquila Section, L’Aquila, Italy.

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Authors and Affiliations

  1. Chair of Hematology, Department of Internal Medicine and Public Health, University of L’Aquila, Via Vetoio snc, Coppito, 67100, L’Aquila, Italy

    Marianna Colagrande, Mauro Di Ianni, Mario Lapecorella & Antonio Tabilio

  2. Department of Pathology, Ospedale San Salvatore, L’Aquila, Italy

    Gino Coletti

  3. Department of Dermatology, University of L’Aquila, L’Aquila, Italy

    Ketty Peris & Maria Concetta Fargnoli

  4. Department of Clinical and Experimental Medicine, Hematology and Clinical Immunology Section, University of Perugia, Perugia, Italy

    Lorenzo Moretti

Authors
  1. Marianna Colagrande
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  2. Mauro Di Ianni
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  3. Gino Coletti
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  4. Ketty Peris
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  5. Maria Concetta Fargnoli
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  6. Lorenzo Moretti
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  7. Mario Lapecorella
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  8. Antonio Tabilio
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Corresponding author

Correspondence to Mauro Di Ianni.

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Colagrande, M., Di Ianni, M., Coletti, G. et al. Toxic epidermal necrolysis in a patient with primary myelofibrosis receiving thalidomide therapy. Int J Hematol 89, 76–79 (2009). https://doi.org/10.1007/s12185-008-0220-2

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  • DOI: https://doi.org/10.1007/s12185-008-0220-2

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