Osteoporosis on systemic mastocytosis: case report
Authors:
Vaňuga Peter 1; Ságová Ivana 1; Kulich Michal 1; Vaňuga Anton 1,2
Authors‘ workplace:
Endokrinologické oddelenie, Národný endokrinologický a diabetologický ústav n. o. (NEDÚ), Ľubochňa
1; Alpha medical, s. r. o., Martin
2
Published in:
Clinical Osteology 2020; 25(2): 85-91
Category:
Overview
Mastocytóza je raritné ochorenie, ktoré je charakterizované abnormálnou proliferáciou mastocytov, so širokým spektrom klinických prejavov v dôsledku postihnutia rôznych tkanív a orgánov. Častou manifestáciou systémovej mastocytózy, najmä v dospelosti, je osteoporóza. Najčastejším miestom postihnutia je lumbálna chrbtica, v dôsledku väčšej straty trabekulárnej kosti v porovnaní s kortikálnou kosťou. Patomechanizmus vzniku osteoporózy pri mastocytóze je multifaktoriálny. K osteoporóze dochádza jednak v dôsledku neoplastickej infiltrácie kostnej drene mastocytmi, najmä však vplyvom uvoľnených mediátorov z mastocytu ako napr. histamínu, heparínu, prostaglandínov, cytokínov, ktoré zvyšujú aktivitu osteoklastov, a tým aj kostnú resorpciu. V liečbe osteoporózy pri mastocytóze sú indikované najmä bisfosfonáty, ďalšou alternatívou je denosumab. V našej kazuistike popisujeme prípad 33-ročnej pacientky s anamnézou urticaria pigmentosa, s ťažkou manifestnou osteoporózou s kompresívnymi fraktúrami torakálnej a lumbálnej chrbtice na podklade agresívnej formy systémovej mastocytózy.
Keywords:
osteoporosis – bisphosphonates – Bone resorption – denosumab – systemic mastocytosis
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Clinical biochemistry Paediatric gynaecology Paediatric radiology Paediatric rheumatology Endocrinology Gynaecology and obstetrics Internal medicine Orthopaedics General practitioner for adults Radiodiagnostics Rehabilitation Rheumatology Traumatology OsteologyArticle was published in
Clinical Osteology
2020 Issue 2
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