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Joint Damage in Non-severe Hemophilia A – Preliminary Results from an Explorative Study

1Amsterdam UMC, University of Amsterdam, Pediatric Hematology, Amsterdam, the Netherlands, 2Amsterdam UMC, University of Amsterdam, Radiology, Amsterdam, the Netherlands, 3Erasmus University Medical Center, Hematology, Rotterdam, the Netherlands, 4Erasmus University Medical Center, Pediatric Hematology, Rotterdam, the Netherlands, 5Amsterdam UMC, University of Amsterdam, Vascular Medicine, Amsterdam, the Netherlands, 6Sanquin Research, Molecular Cellular Hemostasis, Amsterdam, the Netherlands

Abstract Number: PB1015

Meeting: ISTH 2020 Congress

Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Clinical

Background: Joint bleeds are the hallmark of severe hemophilia and may give rise to development of hemophilic arthropathy. In non-severe hemophilia, hemarthroses generally occur less often. However, it has been hypothesized that even subclinical joint bleeds may lead to joint deformation. To date, the extent of joint damage in non-severe hemophilia is unknown.

Aims: To describe the joint status in a cohort of non-severe hemophilia A patients.

Methods: The ongoing DYNAMO study evaluates joint status in non-severe hemophilia A patients (FVIII 2-35 IU/dL, age 24-55 years) at two time points two years apart. Elbows, knees and ankles are assessed by use of the Haemophilia Joint Health Score (HJHS) and by magnetic resonance imaging (MRI) on the same day. MRI’s are reviewed by a musculoskeletal radiologist blinded to clinical data. The study was approved by ethics committees and written consent was obtained (ClinicalTrials.gov Identifier: NCT03623295).

Results: We included 27 non-severe hemophilia A patients with a median age of 43 years (IQR 30-51) and a median FVIII of 8 IU/dL (IQR 5-14). The median HJHS score was 3 out of 124 (IQR 2-6). The MRI IPSG score was calculated from 95 joints in 17 patients (7 joints were not evaluated). The median IPSG score was 5 out of 102 (IQR 2.5-10.0). Lifetime and 10-year bleeding history was retrospectively collected for 15 and 11 patients, respectively. Eight patients had a positive lifetime history of hemarthrosis. The median annual joint bleed rate was 0 (IQR 0.0-0.1). MR imaging demonstrated that ankles (26/33 joints) were more frequently affected than knees (18/34 joints) and elbows (5/28 joints). The most frequent findings over 33 ankle joints were hemosiderin (48%), effusion (42%), cartilage degradation (39%) and synovial hypertrophy (33%). Further analyses will be presented at ISTH 2020.

Conclusions: A substantial part of non-severe hemophilia patients in this cohort has minor joint damage.

To cite this abstract in AMA style:

Zwagemaker A-, Kloosterman FR, Hemke R, Romano LGR, Kruip MJHA, Cnossen MH, Leebeek FWG, Coppens M, Gouw SC, Maas M, Fijnvandraat K, DYNAMO Study Group . Joint Damage in Non-severe Hemophilia A – Preliminary Results from an Explorative Study [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/joint-damage-in-non-severe-hemophilia-a-preliminary-results-from-an-explorative-study/. Accessed September 24, 2023.

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