Contributions
Abstract: O02
Type: Oral presentation
Session Topic: Inherited and acquired coagulation disorders
Presentation during EHA Scientific Conference on Bleeding Disorders:
On Thursday, September 15, 2016 from 09:00 - 10:30
Location: Rubí + Zafir
Background
Recurrent joint bleeds are the main cause of joint deterioration (hemophilia arthropathy) in patients with hemophilia. It is unknown to what extent arthropathy occurs following joint bleeds in patients with Von Willebrands disease compared to hemophilia.
Aims
The primary objective was to compare joint outcome by physical examination between adult patients with VWD and moderate and severe hemophilia A (HA) and a history of joint bleeds. The secondary objective was to compare radiological joint damage between these groups.
Methods
Patients with VWD (VWF activity <30%) or moderate or severe HA, who had a medical history of treatment for joint bleeds, were selected for this preliminary analysis. To compare joint outcome we used the Hemophilia Joint Health Score (HJHS range 0-124, obtained by physical examination) and X-ray Pettersson scores (PS range 0-13 per joint) of ankles, knees and elbows. Data on HA were derived from three previous studies. Univariate analyses were performed using Mann Whitney U and Chi2. For multivariate analysis we performed negative binomial regression analysis (HJHS) and logistic regression (dichotomized PS>3).
Results
47 pts with VWD (mean age 45 yrs) were compared to 36 patients with moderate HA (mean age 38 yrs) and 59 patients with severe HA (mean age 26 yrs). More than 5 joint bleeds had occurred more often in the HA patients (27/48 VWD vs. 30/39 moderate HA vs. 58/59 severe HA, p<0.001). Joint dysfunction at physical examination was comparable between the patients with moderate HA and VWD (median HJHS 5 in VWD, compared to 5.5 in moderate HA, p=0.60 adjusted for age) and slightly worse in severe HA (median HJHS 9, p=0.02 compared to VWD and adjusted for age). In moderate HA insufficient X rays were available for the analysis. Apparent joint damage on X rays (PS>3) occurred significantly more often in severe HA compared to VWD (joints with PS>3: 27/40 severe HA vs. 12/46 VWD, OR 11, 95%CI 3-40, p<0.001 adjusted for age).
Conclusions
Joint function according to the HJHS in patients with a history of treatment for joint bleeds was comparable between patients with VWD and moderate HA but slightly worse in those with severe HA. Patients with severe HA more often had apparent X ray joint damage. Knowledge of similarities and differences in joint outcome between VWD and hemophilia can be helpful to improve the awareness and treatment of joint bleeds in VWD to prevent arthropathy.
These preliminary data have not been published or presented before.
Abstract: O02
Type: Oral presentation
Session Topic: Inherited and acquired coagulation disorders
Presentation during EHA Scientific Conference on Bleeding Disorders:
On Thursday, September 15, 2016 from 09:00 - 10:30
Location: Rubí + Zafir
Background
Recurrent joint bleeds are the main cause of joint deterioration (hemophilia arthropathy) in patients with hemophilia. It is unknown to what extent arthropathy occurs following joint bleeds in patients with Von Willebrands disease compared to hemophilia.
Aims
The primary objective was to compare joint outcome by physical examination between adult patients with VWD and moderate and severe hemophilia A (HA) and a history of joint bleeds. The secondary objective was to compare radiological joint damage between these groups.
Methods
Patients with VWD (VWF activity <30%) or moderate or severe HA, who had a medical history of treatment for joint bleeds, were selected for this preliminary analysis. To compare joint outcome we used the Hemophilia Joint Health Score (HJHS range 0-124, obtained by physical examination) and X-ray Pettersson scores (PS range 0-13 per joint) of ankles, knees and elbows. Data on HA were derived from three previous studies. Univariate analyses were performed using Mann Whitney U and Chi2. For multivariate analysis we performed negative binomial regression analysis (HJHS) and logistic regression (dichotomized PS>3).
Results
47 pts with VWD (mean age 45 yrs) were compared to 36 patients with moderate HA (mean age 38 yrs) and 59 patients with severe HA (mean age 26 yrs). More than 5 joint bleeds had occurred more often in the HA patients (27/48 VWD vs. 30/39 moderate HA vs. 58/59 severe HA, p<0.001). Joint dysfunction at physical examination was comparable between the patients with moderate HA and VWD (median HJHS 5 in VWD, compared to 5.5 in moderate HA, p=0.60 adjusted for age) and slightly worse in severe HA (median HJHS 9, p=0.02 compared to VWD and adjusted for age). In moderate HA insufficient X rays were available for the analysis. Apparent joint damage on X rays (PS>3) occurred significantly more often in severe HA compared to VWD (joints with PS>3: 27/40 severe HA vs. 12/46 VWD, OR 11, 95%CI 3-40, p<0.001 adjusted for age).
Conclusions
Joint function according to the HJHS in patients with a history of treatment for joint bleeds was comparable between patients with VWD and moderate HA but slightly worse in those with severe HA. Patients with severe HA more often had apparent X ray joint damage. Knowledge of similarities and differences in joint outcome between VWD and hemophilia can be helpful to improve the awareness and treatment of joint bleeds in VWD to prevent arthropathy.
These preliminary data have not been published or presented before.