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Systemic Mastocytosis and Essential Thrombocythemia: Case Report and Literature Overview

Mauro Cancian, Elisabetta Cosi, Marco Pizzi, Sandro Giannini, Irene Bertozzi, Fabrizio Fabris, Maria Luigia Randi
Published: 25 August 2019
 by  MDPI
Medicina , Volume 55; doi:10.3390/medicina55090528

Abstract: Mastocytosis is a rare disease in which heightened amounts of mast cells accumulate in the skin, bone marrow, and other visceral organs. Upon activation, mast cells release a wide variety of preformed or newly synthesized mediators which can induce allergic symptoms and inflammatory reactions. Mastocytosis is diagnosed by biopsy and can be divided into cutaneous and systemic mastocytosis (SM). The first one affects the skin and is relatively benign, whilst SM, which involves bone marrow and other organs, may be aggressive and associate with both myelodisplastic and myeloproliferative diseases. Here we present a case of SM associated with essential thrombocythemia and complicated by severe osteoporosis, successfully treated with hydroxyurea, low-dose aspirin and zolendronic acid.
Keywords: hydroxyurea / Osteoporosis / mastocytosis / thrombocythemia / Zolendronic Acid

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