Abstract Number: PB0533
Meeting: ISTH 2021 Congress
Background: Osteopenia and osteoporosis leading to increase rate of fractures are now well recognised comorbidities in haemophilia patients. The pathogenesis is multifactorial and includes decreased thrombin generation as a factor of impaired osteoblastic differentiation as well as increased osteoclastic activity.
Aims: To evaluate BMD of severe haemophilia A adult patients and to assess possible correlation between serum levels of circulating remodeling biomarkers -OPG,RANKL ,CTX ,osteocalcin, sclerostin and VitD level.
Methods: 26 male subjects (median age 42,88±11,73 with BMI 24,86±3,59) affected with severe haemophilia A were enrolled .Patients with chronic liver disease and HCV were excluded. In addition 33 age -matched controls(median age 38,69±12,69 with BMI 22,61±2,84) were recruited. All subjects provided written informed consent and all procedures were approved by local review board. Biomarkers of bone metabolism were tested with ELISA assay and BMD was investigated with DEXA of two zones -lumbar spine and total hip. Data was analysed statistically using SPSS package.
Results: Patients with haemophilia had lower LS-BMD – 0.955 ± 0,145 vs 1,118 ± 0,079,p=0,05 and TH-BMD – 0,840 ± 0,147 vs 0,951 ± 0,075 ,p=0,05 comparing with controls ,respectively TH T-score of -1,41±0,91 vs 0,4 ± 0,49 ,p=0,05 and LS-T-score of -1,16 ± 1,046 vs 0,14 ± 0,72,p=0,05. 66,6% of patients <50 years had osteopenia and 8,3% -osteoporosis. Among those >50 years 50% experience osteopenia and 20% osteoporosis. They had significantly higher OPG (123,69 ± 107,05 vs 41,98 ± 18,95,p=0,05) and lower sRANKL(23,49 ± 29,39 vs 131,32 ± 201,27,p=0,05) levels ,sRANKL/OPG ratio 0,27± 0,35 vs 5,28 ± 10,01 ,p=0,05 with positive correlation between sRANKL and BMD and T score of lumbar spine,p=0,001.
Conclusions: Higher circulating osteoprotegerin levels in hemophilia patients with osteopenia/osteoporosis are reflection of increased bone turnover and a compensatory response to excessive osteoclast activity. Our results suggest that sRANKL level and ratio can be used as a predictor of BMD but further investigation will be necessary.
To cite this abstract in AMA style:Ivanova H, Grudeva-Popova Z, Deneva T, Mateva N. A single center study of bone mineral density in adult patients with severe Haemophilia A and correlation with markers of bone metabolism [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/a-single-center-study-of-bone-mineral-density-in-adult-patients-with-severe-haemophilia-a-and-correlation-with-markers-of-bone-metabolism/. Accessed October 1, 2023.
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