Abstract Number: PB0476
Meeting: ISTH 2021 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Basic
Background: It has been observed that patients with hemophilia have a tendency to have low bone mineral density (BMD). It is generally accepted that the etiology is the low physical activity due to the hemophilic arthropathy that they suffer. Several studies have recently proposed that the deficiency of factor VIII is independently correlated with the reduction of the BMD, even in the absence of hemorrhage and hemophilic arthropathy.
Aims: To assess the BMD in children regarding the treatment of the patients, and to determine the difference of BMD between patients with moderate and severe hemophilia.
Methods: This is an observational, descriptive, cross-sectional pilot study in which we included a total of 22 patients between ages 3 to 15. Patients were divided into 2 groups: severe and moderate hemophilia. Each patient was submitted to a bone densitometry and they were compared to 68 controls from a historic database.
Results: Seventeen patients had severe hemophilia and five were moderate. The spine Z-Score in the moderate group was -1.8800 and in the severe group was -0.4471 with a p value of 0.008. Four patients receiving on demand therapy were compared with the control group, with a mean hip Z-Score of -2.10 and -0.77 respectively, with a p value of 0.032.
Conclusions: We found that the spine Z-Score of patients with severe hemophilia was significantly higher than in the moderate group (p=0.008) (Table 1). We also observed that patients that were on demand therapy had significantly lower hip Z-Score than healthy children (Table 2). No correlation could be demonstrated between the amount of factor VIII and low bone mineral density in children with hemophilia. These results should be further complemented with a larger sample size. This suggests the possibility that the lack of FVIII alone may not be responsible for BMD reduction.
Table 1
| Moderate n=5 Mean (SD) |
Severe n=17 Mean (SD) |
P value | |
| Hip Z-Score | -1.84 (1.193) | -1.15 (1.214) | 0.324 |
| Spine Z-Score | -1.8800 (0.8318) | -0.4471 (0.8063) | 0.008* |
Comparison of densitometry testing between moderate and severe hemophilia.
Table 2
| Demand n=4 Mean (SD) |
Control n=68 Mean (SD) |
P value | |
| Hip Z Score | -2.10 (1.445) | -0.77 (1.157) | 0.032 |
| Spine Z Score | -0.6000 (1.4159) | -0.5662 (1.0676) | 0.947 |
Comparison of densitometry testing between patients with on demand therapy and controls.
To cite this abstract in AMA style:
Galindo-Aguilar OU, García-Hernández PA, Garza JÁ, Garay-Mendoza D, Ortiz-Castillo M, González-Martínez A, Bautista-Gómez AJ, González-Martínez G, Molina-de la Garza JF, Villarreal Martínez L. Association between Factor VIII Deficiency and Low Bone Mineral Density in Children with Hemophilia [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/association-between-factor-viii-deficiency-and-low-bone-mineral-density-in-children-with-hemophilia/. Accessed December 8, 2021.« Back to ISTH 2021 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/association-between-factor-viii-deficiency-and-low-bone-mineral-density-in-children-with-hemophilia/