Thromb Haemost 2022; 122(05): 692-702
DOI: 10.1055/s-0041-1735964
Coagulation and Fibrinolysis

The Bone Microarchitecture Deficit in Patients with Hemophilia Is Influenced by Arthropathy, Hepatitis C Infection, and Physical Activity

Katharina Holstein*
1   Department of Haematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Leonora Witt*
1   Department of Haematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Anna Matysiak
1   Department of Haematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Constantin Schmidt
2   Department of Osteology and Biomechanics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
3   Department of Trauma and Orthopaedic Surgery, Division of Orthopaedics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Florian Barvencik
2   Department of Osteology and Biomechanics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Michael Amling
2   Department of Osteology and Biomechanics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Tim Rolvien*
3   Department of Trauma and Orthopaedic Surgery, Division of Orthopaedics, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Florian Langer*
1   Department of Haematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
› Author Affiliations

Abstract

Low bone mineral density (BMD) is common in patients with hemophilia (PWHs). The aim of the present study was to describe BMD and microarchitecture in PWHs in Northern Germany and to determine factors contributing to possible skeletal alterations. Demographic characteristics, BMD and microarchitecture, bone metabolism markers, and orthopaedic joint score (OJS) were assessed during routine check-ups. Areal BMD was assessed by dual-energy X-ray absorptiometry (DXA) at the hip and lumbar spine. Volumetric BMD and microarchitecture were quantified by high-resolution peripheral quantitative computed tomography at the distal radius and tibia. Eighty male PWHs (median age, 33 years; range, 18–77) were retrospectively analyzed, of whom 67 (84.0%) and 13 (16.0%) had hemophilia A and B, respectively. Fifty-four (68.0%), six (7.0%), and 20 (25.0%) patients had severe, moderate, or mild hemophilia, and 35 (44.0%) were hepatitis C virus (HCV) positive. DXA analysis revealed low BMD (Z-score ≤ − 2.0) in 27.5% of PWHs, and higher bone turnover values were associated with lower BMD. Bone microarchitecture was dominated by cortical deficits at the radius and trabecular deficits at the tibia. Cortical deficits at the radius were influenced by lower body mass index, low-grade inflammation, and treatment regimen (higher cortical thickness on primary prophylaxis). Trabecular alterations at the tibia were mainly associated with OJS and HCV status. A positive effect of self-reported sportive activity on BMD could be shown. In conclusion, our findings demonstrate that the site-specific microarchitectural deficit observed in PWHs is primarily negatively influenced by poor joint status, inflammation, HCV infection, and high bone turnover.

Author Contributions

K.H., L.W., M.A., F.B., and F.L. designed the study; K.H., L.W., A.M., and C.S. enrolled patients and collected data; K.H., L.W., and T.R. analyzed the data and wrote the first draft of the manuscript; all authors contributed to data interpretation, manuscript writing, and approved the final version of the manuscript.


* These authors contributed equally to this study.


Supplementary Material



Publication History

Received: 22 December 2020

Accepted: 17 August 2021

Article published online:
29 September 2021

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