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
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