C L Kempton1, D M Antoniucci2, E C Rodriguez-Merchan3,4. 1. Department of Pediatrics and Department of Hematology and Medical Oncology, Emory University School of Medicine, Atlanta, GA, USA. 2. Division of Endocrinology Sutter Pacific Medical Foundation, Department of Medicine University of California, San Francisco, CA, USA. 3. Department of Orthopaedic Surgery, La Paz University Hospital, Madrid, Spain. 4. School of Medicine, Autonomous University, Madrid, Spain.
Abstract
INTRODUCTION: As the population of patients with haemophilia (PWH) ages, healthcare providers are required to direct greater attention to age-related co-morbidities. Low bone mineral density (BMD) is one such co-morbidity where the incidence not only increases with age, but also occurs with greater frequency in PWH. AIM: To review risk factors for low BMD, and strategies to promote bone health and identify patients who would benefit from screening for osteoporosis and subsequent treatment. METHODS: A narrative review of the literature was performed in MEDLINE with keywords haemophilia, bone density, osteoporosis and fracture. Reference lists of retrieved articles were also reviewed. RESULTS: Low BMD occurs more commonly in PWH than the general population and is most likely the result of a combination of risk factors. Steps to promote bone health include preventing haemarthrosis, encouraging regular exercise, adequate vitamin D and calcium intake, and avoiding tobacco and excessive alcohol intake. Adults 50 years of age and older with haemophilia and those younger than 50 years with a fragility fracture or increased fracture risk based on FRAX (The Fracture Risk Assessment Tool), regardless of haemophilia severity, should be screened for low BMD using dual x-ray absorptiometry (DXA). Once osteoporosis is diagnosed based on DXA, fracture risk should guide treatment. Currently, treatment is similar to those without haemophilia and most commonly includes bisphosphonates. CONCLUSION: Haemophilia care providers should promote adequate bone formation during childhood and reduce bone loss during adulthood as well as identify patients with low BMD that would benefit from therapy.
INTRODUCTION: As the population of patients with haemophilia (PWH) ages, healthcare providers are required to direct greater attention to age-related co-morbidities. Low bone mineral density (BMD) is one such co-morbidity where the incidence not only increases with age, but also occurs with greater frequency in PWH. AIM: To review risk factors for low BMD, and strategies to promote bone health and identify patients who would benefit from screening for osteoporosis and subsequent treatment. METHODS: A narrative review of the literature was performed in MEDLINE with keywords haemophilia, bone density, osteoporosis and fracture. Reference lists of retrieved articles were also reviewed. RESULTS: Low BMD occurs more commonly in PWH than the general population and is most likely the result of a combination of risk factors. Steps to promote bone health include preventing haemarthrosis, encouraging regular exercise, adequate vitamin D and calcium intake, and avoiding tobacco and excessive alcohol intake. Adults 50 years of age and older with haemophilia and those younger than 50 years with a fragility fracture or increased fracture risk based on FRAX (The Fracture Risk Assessment Tool), regardless of haemophilia severity, should be screened for low BMD using dual x-ray absorptiometry (DXA). Once osteoporosis is diagnosed based on DXA, fracture risk should guide treatment. Currently, treatment is similar to those without haemophilia and most commonly includes bisphosphonates. CONCLUSION:Haemophilia care providers should promote adequate bone formation during childhood and reduce bone loss during adulthood as well as identify patients with low BMD that would benefit from therapy.
Authors: Coline Haxaire; Narine Hakobyan; Tania Pannellini; Camila Carballo; David McIlwain; Tak W Mak; Scott Rodeo; Suchitra Acharya; Daniel Li; Jackie Szymonifka; Xiangqian Song; Sébastien Monette; Alok Srivastava; Jane E Salmon; Carl P Blobel Journal: Blood Date: 2018-05-18 Impact factor: 22.113
Authors: Sarah Taves; Junjiang Sun; Eric W Livingston; Xin Chen; Jerome Amiaud; Regis Brion; William B Hannah; Ted A Bateman; Dominique Heymann; Paul E Monahan Journal: Sci Rep Date: 2019-10-08 Impact factor: 4.379