Osteoporosis in systemic mastocytosis — current therapeutic options based on a clinical case report
Abstract
Systemic mastocytosis (SM) is a rare hematologic neoplastic disorder characterized by infiltration of various organs by clonal mast cells. It s characterized with cutaneous and organ involvement (hepatosplenomegaly, osteolytic changes, pathological fractures) and systemic symptoms related to the release of anaphylaxis mediators. Systemic mastocytosis was diagnosed in a 50-year-old patient based on WHO criteria. The disease course included fractures of the TH11 and L1 vertebrae, and densitometric tests revealed low bone mineral density (BMD). The patient was treated with risedronate for 3 years, yet BMD continued to decline. The therapy was switched to intravenous zoledronic acid, resulting in a significant increase in BMD in control tests and relief from pain. No new osteoprotic fractures were observed during the treatment.
Keywords: mastocytosisosteoporosistreatmentbisphosphonates
References
- Amon U, Hartmann K, Horny HP, et al. Mastocytosis - an update. J Dtsch Dermatol Ges. 2010; 8(9): 695–711; quiz 712.
- Rossini M, Zanotti R, Orsolini G, et al. Prevalence, pathogenesis, and treatment options for mastocytosis-related osteoporosis. Osteoporos Int. 2016; 27(8): 2411–2421.
- Valent P, Akin C, Escribano L, et al. Standards and standardization in mastocytosis: consensus statements on diagnostics, treatment recommendations and response criteria. Eur J Clin Invest. 2007; 37(6): 435–453.
- Valent P, Akin C, Sperr WR, et al. New insights into the pathogenesis of mastocytosis: emerging concepts in diagnosis and therapy. Annu Rev Pathol. 2023; 18: 361–386.
- Sciumè M, De Magistris C, Galli N, et al. Target therapies for systemic mastocytosis: an update. Pharmaceuticals (Basel). 2022; 15(6): 738.
- Makovoz A, Wang J, Oshegbo G, et al. Assessment of osteoporosis and fracture risk in mastocytosis within a North American cohort. J Allergy Clin Immunol Pract. 2021; 9(12): 4459–4467.e10.
- Gaudio A, Xourafa A, Rapisarda R, et al. Hematological diseases and osteoporosis. Int J Mol Sci. 2020; 21(10): 3538.
- Pardanani A, Reichard K, Tefferi A. Advanced systemic mastocytosis - revised classification, new drugs and how we treat. Br J Haematol. 2024; 204(2): 402–414.
- El Hussein S, Chifotides HT, Khoury JD, et al. Systemic mastocytosis and other entities involving mast cells: a practical review and update. Cancers (Basel). 2022; 14(14).
- Rossini M, Zanotti R, Viapiana O, et al. Bone involvement and osteoporosis in mastocytosis. Immunol Allergy Clin North Am. 2014; 34(2): 383–396.
- Pardanani A. Systemic mastocytosis in adults: 2023 update on diagnosis, risk stratification and management. Am J Hematol. 2023; 98(7): 1097–1116.
- Zanotti R, Tanasi I, Crosera L, et al. Systemic mastocytosis: multidisciplinary approach. Mediterr J Hematol Infect Dis. 2021; 13(1): e2021068.
- Renke J, Kędzierska-Mieszkowska S, Lange M, et al. Mast cells in mastocytosis and allergy - Important player in metabolic and immunological homeostasis. Adv Med Sci. 2019; 64(1): 124–130.
- Sirufo MM, Suppa M, Ginaldi L, et al. Does allergy break bones? Osteoporosis and its connection to allergy. Int J Mol Sci. 2020; 21(3): 712.
