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Clinical Trial
. 2018 Aug 28;2(16):2136-2144.
doi: 10.1182/bloodadvances.2018020552.

The frequency of joint hemorrhages and procedures in nonsevere hemophilia A vs B

J Michael Soucie  1 , Paul E Monahan  2   3 , Roshni Kulkarni  4 , Barbara A Konkle  5   6 , Marshall A Mazepa  7 ; US Hemophilia Treatment Center Network
Affiliations
Clinical Trial

The frequency of joint hemorrhages and procedures in nonsevere hemophilia A vs B

J Michael Soucie et al. Blood Adv. .

Abstract

Data are needed on minimal factor activity (FA) levels required to prevent bleeding in hemophilia. We aimed to evaluate associations between hemophilia type and FA level and joint bleeding and orthopedic procedures using longitudinal data. Data were collected over an 11-year period on males with nonsevere hemophilia A or B without inhibitors who were receiving on-demand factor replacement therapy. Data on the number of joint bleeds in the previous 6 months and data on procedures from clinical records were analyzed using regression models. Data were collected on 4771 patients (hemophilia A, 3315; hemophilia B, 1456) from 19 979 clinic visits. Ages ranged from 2 to 91 years and baseline FA level ranged from 1% to 49% with a mean of 9.4%. Joint bleeding rates were heterogeneous across the FA range and were highest among men age 25 to 44 years. Adjusted for FA level, the mean number of joint bleeds per 6 months was 1.4 and 0.7 for patients with hemophilia A and B, respectively (P < .001). Regression models predicted 1.4 and 0.6 bleeds per year for hemophilia A and B patients, respectively, at an FA level of 15%. Patients with hemophilia B were 30% less likely than those with hemophilia A to have undergone an orthopedic procedure. We conclude that joint bleed rates for any given FA level were higher among hemophilia A than hemophilia B patients, and target FA levels of 15% are unlikely to prevent all joint bleeding in US males with hemophilia.

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Conflict of interest statement

Conflict-of-interest disclosure: B.A.K. received consulting fees from Bioverativ, CSL Behring, Genentech, and Shire; and research support from Bioverativ, Sangamo, and Shire. P.E.M. received research support through the University of North Carolina from Asklepios, Novo Nordisk, and Baxter Healthcare; he holds patents that have been licensed to Asklepios for which he receives royalties; has received payment for consultation, services, and for speaking for Asklepios, Chatham LLC, Baxter Healthcare, Pfizer, Bayer, Novo Nordisk, and Biogen; and was employed by Shire during drafting of the manuscript. The remaining authors declare no competing financial interests.

Figures

None
Graphical abstract
Figure 1.
Figure 1.
Distribution of average number of joint bleeds across FA level by age groups for patients with hemophilia A. In the boxplots, the central rectangle spans the first quartile to the third quartile, a horizontal line inside the rectangle shows the median, and a circle shows the mean. Vertical lines that extend to the top of the graph indicate the presence of outlier values above 10 joint bleeds per 6-month period.
Figure 2.
Figure 2.
Distribution of average number of joint bleeds across factor activity level by age groups for patients with hemophilia B. In the boxplots, the central rectangle spans the first quartile to the third quartile, a horizontal line inside the rectangle shows the median, and a circle shows the mean. Vertical lines that extend to the top of the graph indicate the presence of outlier values above 6 joint bleeds per 6-month period.
Figure 3.
Figure 3.
Predicted number of joint bleeds according to factor activity level and age group for patients with hemophilia A or B based on a regression model. Comparison of predicted number of joint bleeds per 6-month period according to FA level for a hypothetical white patient with commercial insurance, normal BMI, and HIV negativity with either hemophilia A (dashed line) or hemophilia B (solid line).
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