Several predisposing risk factors exist for decreased bone mineral density in patients with hemophilia increasing the likelihood of osteopenia and osteoporosis in this population. We report the findings of a study evaluating the association of vitamin D deficiency, osteoporosis and hemophilia.
Describe the findings of a prospective study evaluating vitamin D levels and bone mineral density in patients with hemophilia. We hypothesize that males with hemophilia have decreased vitamin D levels increasing their risk of osteoporosis along with arthropathy and periods of immobility resulting in reduced bone loading.
Males with hemophilia, age 1-66 years, were eligible for a 3 part study including: 1) data collection on basic health; 2) laboratory measurement of 25-OH Vitamin D, liver enzymes, ionized calcium, magnesium, phosphorus, parathyroid hormone (PTH), and thyroid function tests; and 3) DEXA scan.
86 male subjects, age 2-64 years (mean: 19.9, median: 15.0) including 69 (80%) with hemophilia A and 17 (20%) with hemophilia B were enrolled along with 9 (10%) with history of an inhibitor. Vitamin D levels were reduced in 51 (65%) subjects, including 23 (30%) who were vitamin D insufficient (20-29 ng/mL) and 28 (36%) who were vitamin D deficient (<20 ng/mL). Of the 8 enrolled subjects with history of inhibitor who have had vitamin D levels tested, 7 (88%) are abnormally low with range 17-30 ng/mL for all 8. Among the 61 DEXA scans completed, 8 (13%) were abnormal resulting in a diagnosis of osteoporosis – 6 adults and 2 children (age range 12-56 years). The 2 children with abnormal DEXA scans have a history of inhibitor; the 6 adults are all infected with HIV and/or HCV. 7 of the 8 subjects with osteoporosis had vitamin D levels drawn, where 3 (43%) were abnormally low, with a range of only 10-33 ng/mL among all 7.
Among hemophilia patients enrolled in this study, 65% have abnormally low vitamin D levels which may predispose to decreased bone mineral density over their lifetime. Patients with a history of inhibitor appear to have an increased risk of reduced vitamin D levels. Vitamin D and calcium supplementation may be a simple and inexpensive intervention to address this complication and warrants further investigation in this population. 13% of enrolled subjects had osteoporosis on DEXA where a history of inhibitor or coinfection with HIV and/or HCV is prevalent. High risk subjects may deserve routine screening to allow for earlier intervention.
Funding:CSL Behring Foundation grant
Valentino:Baxter: Consultancy, Membership on an entity’s Board of Directors or advisory committees; Bayer: Consultancy, Membership on an entity’s Board of Directors or advisory committees; Biogen Idec: Consultancy, Membership on an entity’s Board of Directors or advisory committees; GTC Biotherapeutics: Consultancy, Membership on an entity’s Board of Directors or advisory committees; Inspiration: Consultancy, Membership on an entity’s Board of Directors or advisory committees; Novo Nordisk: Consultancy, Membership on an entity’s Board of Directors or advisory committees.
Asterisk with author names denotes non-ASH members.