Systemic Mastocytosis and Essential Thrombocythemia: Case Report and Literature Overview
Open Access
- 25 August 2019
- Vol. 55 (9), 528
- https://doi.org/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
This publication has 16 references indexed in Scilit:
- The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemiaBlood, 2016
- Impact of centralized evaluation of bone marrow histology in systemic mastocytosisEuropean Journal of Clinical Investigation, 2016
- Systemic Mastocytosis: Clinical Update and Future DirectionsClinical Lymphoma Myeloma and Leukemia, 2015
- Myeloproliferative NeoplasmsJAMA Oncology, 2015
- Zoledronic Acid in Osteoporosis Secondary to MastocytosisThe American Journal of Medicine, 2014
- Prevalence of indolent systemic mastocytosis in a Dutch regionJournal of Allergy and Clinical Immunology, 2013
- Survival and Disease Progression in Essential Thrombocythemia Are Significantly Influenced by Accurate Morphologic Diagnosis: An International StudyJournal of Clinical Oncology, 2011
- Prognostically relevant breakdown of 123 patients with systemic mastocytosis associated with other myeloid malignanciesBlood, 2009
- SYSTEMIC MASTOCYTOSIS AND PRIMARY THROMBOCYTHAEMIABritish Journal of Haematology, 1991