Introduction

Immune thrombocytopenic purpura (ITP) is an acquired thrombocytopenia that leads to clinically significant bleeding in many. The Bleeding Assessment Tool (BAT) is a validated tool used to assess the degree of bleeding with scoring directly proportional to the severity of bleeding. Predicting the occurrence of bleeding is challenging in patients with ITP. Hypermobility syndromes have been associated with higher rates of clinically significant bleeding. Joint hypermobility, assessed by the Beighton Scoring System, has been shown to be associated with more severe bleeding phenotypes in persons with hemophilia, von Willebrand disease, and in some platelet function disorders, but has not been studied in patients with ITP.

Methods

We conducted a retrospective observational cohort study to investigate the relationship between joint hypermobility and the degree of bleeding by comparing BAT scores and age/sex-specific Beighton scores in patients with ITP at least 6 years age at the Bleeding and Clotting Disorders Institute. Correlations were determined using nonparametric analyses to test the difference between two groups: (a) abnormal BAT versus Beighton scores and (b) abnormal BAT versus degree of thrombocytopenia or sex. The Independent-Samples Kruskal-Wallis Test was used to determine the impact of age and platelet count on BAT and Beighton scores, if one existed.

Results

Data from 174 patients were obtained, though 122 were excluded due to a lack of BAT or Beighton score, or both. Of the 52 patients remaining, 34 (65.4%) were female and 18 (34.6%) were male, with ages ranging between 12-81 years. BAT scores ranged between 0-18 with a median of 5 and mean of 5.8+4.4, while Beighton scores ranged between 0-9 with a median of 3.5 and mean of 3.4+3.4. A moderate correlation (r values between 0.5-0.7) was found between overall BAT and Beighton scores (r=0.66), while an inverse correlation was found between Beighton scores and platelet counts (r= -0.89). All 12 patients with positive Beighton scores had abnormal (high) BAT scores (r=1.00) and were all female. Overall, there was no significant association (p=0.681) between gender, BAT, and Beighton scores. Further, there was no significant difference in BAT and Beighton scores across age and platelet count.

Conclusions

Hypermobility was associated with more bleeding based on higher BAT scores. The degree of bleeding was not predicted by the platelet count or age. Our data also suggest that females with ITP and hypermobility are more likely to have more clinically significant bleeding, however results comparing hypermobility and platelet count across the categories of age and sex were unrevealing. Broader investigation is needed to determine the full impact of the association of joint hypermobility and bleeding severity in patients with ITP.

Disclosures

Tarantino:Amgen: Consultancy, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Genentech: Consultancy, Research Funding; Novo Nordisk: Consultancy; Novartis: Consultancy; Octapharma: Consultancy, Research Funding; Sanofi: Consultancy, Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau; Sobi: Consultancy, Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau; Takeda: Consultancy, Research Funding.

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