Abstract Number: PB0691
Meeting: ISTH 2021 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Rare Bleeding Disorders
Background: People with unclassified bleeding disorders and mild platelet function disorders are at risk of bleeding when undergoing surgery or invasive dental work. The optimal treatment has not been established.
Aims: The aim of this study was to assess changes in platelet function, Von Willebrand Factor (VWF) and Factor VIII for patients with platelet function disorders and unclassified bleeding disorders following a desmopressin challenge and to assess the incidence of bleeding when they underwent invasive procedures and were treated pre-operatively with desmopressin.
Methods: Participants were included if they were registered at the Centre for Haemostasis and Thrombosis, St Thomas’ Hospital, London, UK, with a diagnosis of unclassified bleeding disorder or platelet function disorder, and had a desmopressin challenge between May 2009 and May 2020.
For desmopressin trials, blood samples were taken at baseline prior to desmopressin 0.3mcg/kg infusion and 30-60 minutes after the infusion. For surgery, desmopressin 0.3mcg/kg subcutaneously 60-90 minutes pre-operatively was given in combination with tranexamic acid 1 gram three times a day. Data were compared using Wilcoxon sign rank test. A p-value <0.05 was considered statistically significant.
Characteristic | Median (IQR) or n (%) | |
Age (years) | 40; 28 to 52 | |
Gender | Male Female |
2 (5%) 40 (95%) |
Family history of bleeding disorder | First degree relative Second degree relative No family history |
24 (57%) 2 (5%) 16 (38%) |
ISTH BAT score | 9.5 (6 to 12) | |
Previous bleeding symptoms | Bleeding after dental extraction Surgical bleeding Menorrhagia Post-partum haemorrhage |
13 (31%) 20 (48%) 39 (98%), n=40 12 (30%), n=40 |
Diagnosis | Unclassified bleeding disorder Platelet function disorder |
20 (48%) 22 (52%) |
Baseline blood tests | VWF antigen (iu/dL) VWF activity (GP1bR) (iu/dL) VWF collagen binding (iu/dL) Factor VIII chromogenic (iu/dL) |
86 (66 to 100), n=41 73 (59 to 89), n=41 68 (58 to 88), n=41 96 (81 to 125), n=39 |
Forty-two patients were included (Table 1). Responses to desmopressin are outlined in Figure 1. Overall VWF antigen increased by 50 to 380% compared to baseline (Figure 1).
23/42 (55%) patients who had a desmopressin challenge, went onto to have surgical procedures and were treated with desmopressin before surgery. The 23 patients underwent 48 invasive procedures: eight tooth extractions, 23 minor surgeries and 17 major surgeries. There were no bleeding complications and no instances of arterial thrombosis, venous thrombosis or hyponatraemic seizures.
Conclusions: Patients with unclassified bleeding disorders and platelet function disorders both had a consistent improvement in VWF levels and reduction in PFA100 closure time with desmopressin challenge. We found no instances of significant bleeding when desmopressin was given pre-operatively.
To cite this abstract in AMA style:
Desborough M, Alamelu J, Austin S, Ling G, Foxton E, McCormick A, Dolan G, Madan B. Unclassified Bleeding Disorders and Inherited Platelet Function Disorders: Response to Desmopressin Challenge [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/unclassified-bleeding-disorders-and-inherited-platelet-function-disorders-response-to-desmopressin-challenge/. Accessed March 22, 2024.« Back to ISTH 2021 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/unclassified-bleeding-disorders-and-inherited-platelet-function-disorders-response-to-desmopressin-challenge/