The Effectiveness of Biophosphonates on Bone involvement in Patients with Systemic Mastocytosis

Onnes, M.C. (Merel) (2016) The Effectiveness of Biophosphonates on Bone involvement in Patients with Systemic Mastocytosis. thesis, Medicine.

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Abstract

Indolent systemic mastocytosis (ISM) is a subtype of mastocytosis that is characterized by a mild proliferation and accumulation of pathological mast cells in extracutaneous tissues, with or without cutaneous involvement. Accumulation and degranulation of mast cells in the bone may cause symptoms of bone involvement, the most important symptoms being fragility fractures and osteoporosis. These patients are generally treated with bisphosphonates, according to the guidelines for the regular, postmenopausal osteoporosis population with increased fracture risk. However, considering the different pathophysiological mechanism causing the symptoms and the different composition of the ISM population compared to the postmenopausal population, questions on the effectiveness of bisphosphonates as a treatment for patients with ISM and osteoporosis and/or fragility fractures can be raised. This study is the largest study to evaluate the effectiveness of bisphosphonates in 50 ISM patients with symptoms of bone involvement with regards to fracture rate, bone mineral density (BMD) and zCTx scores during five years of follow-up. Bisphosphonates significantly increased the lumbar BMD and decreased bone turnover, considering the decrease in zCTx scores. A decrease in fracture rate prior to and after treatment was found when assuming that fractures prior to treatment are detected within two years. Interpretation of this fracture rate is difficult, since a proper control group is lacking. Therefore, final conclusions on the effectiveness of bisphosphonates as a treatment for patients with ISM and bone involvement in the form of fragility fractures and osteoporosis cannot be made. However, considering the large number of fractures in this patient population, proper treatment is necessary and future randomized studies are highly recommended to further evaluate this treatment option.

Item Type:	Thesis (Thesis)
Supervisor name:	Faculty Supervisor: and Oude Elberink, J.N.G. MD/PHD and Department of Allergology and University Medical Center Groningen
Faculty:	Medical Sciences
Date Deposited:	25 Jun 2020 10:45
Last Modified:	25 Jun 2020 10:45
URI:	https://umcg.studenttheses.ub.rug.nl/id/eprint/680

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