Abstract Number: OC 49.4
Meeting: ISTH 2021 Congress
Background: Joint bleeding is the hallmark of hemophilia with the ensuing risk of hemophilic arthropathy. Although overt joint bleeding occurs less frequently in non-severe hemophilia compared to severe hemophilia, subclinical bleeding may occur. It has been suggested that even one overt bleed or subclinical bleeding may lead to joint changes. Currently, little is known about the joint status of patients with non-severe hemophilia A.
Aims: To investigate the extent of joint damage in patients with non-severe hemophilia A.
Methods: The DYNAMO substudy is a cross-sectional study that included non-severe hemophilia A patients (FVIII 2-35 IU/dL) aged 24-55 years. Joint status was assessed with the Haemophilia Joint Health Score (HJHS) and magnetic resonance imaging (MRI) of elbows, knees and ankles (IPSG-score). Joint bleeding data were collected from medical files and calculated as annualized joint bleeding rates (AJBRs) and cumulative joint bleeds over lifetime (all joint bleeds) and over the last 10 years (treated joint bleeds). Correlations between factor level and HJHS/IPSG-score and age were assessed (Spearman’s coefficients). Medical ethics approval and written consent was obtained (ClinicalTrials.gov: NCT03623295).
Results: In total, 52 patients were included with a median age of 43 years (IQR 32-50) and a median FVIII level of 10 IU/dL (IQR 4-16). Forty patients (77%) had a history of hemarthrosis (Table 1).
|Age in years, median (IQR)||43 (38-50)||42 (29-50)||43 (32-50)|
|FVIII level in IU/dL, median (IQR)||2 (2-5)||14 (10-18)||10 (4-16)|
|Zero joint bleeds, n (%)||2 (10)||10 (31)||12 (23)|
|Lifetime cumulative joint bleeds, median (IQR)||9 (2-16)||1 (0-3)||2 (0-7)|
|Lifetime AJBR, median (IQR)||0.3 (0.1-0.4)||0.0 (0.0-0.1)||0.0 (0.0-0.2)|
|Last 10 years cumulative treated joint bleeds, median (IQR)||1 (0-5)||0 (0-1)||0 (0-2)|
|Last 10 years treated AJBR, median (IQR)||0.1 (0.0-0.4)||0.0 (0.0-0.1)||0.0 (0.0-0.2)|
|HJHS score, median (IQR)||7 (3-11)||2 (1-4)||3 (2-7)|
|IPSG score, median (IQR)||7 (2-12)||4 (2-7)||4 (2-9)|
On MRI, soft tissue changes were present in elbows, knees and ankles in 19%, 71% and 70% of patients, respectively. Osteochondral changes were present in elbows, knees and ankles in 0%, 20% and 34% of patients, respectively. In 8 patients, the IPSG-score was ≥3 despite no history of overt joint bleeding. Factor level was not associated with age, negatively associated with HJHS-score and weakly associated with IPSG-score (Figure 1).
Conclusions: In a significant proportion of patients with non-severe hemophilia A joint damage is present despite a low frequency of joint bleeds.
To cite this abstract in AMA style:Kloosterman FR, Zwagemaker A, Hemke R, Gouw SC, Coppens M, Romano LGR, Kruip MJHA, Cnossen M, Leebeek FWG, Maas M, Fijnvandraat K. Joint Status of Patients with Non-severe Hemophilia A [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/joint-status-of-patients-with-non-severe-hemophilia-a/. Accessed November 27, 2021.
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