Abstract Number: PO0041
Meeting: ISTH 2022 Congress
Background: Severe joint degeneration is a common complication in patients suffering from inherited bleeding disorders. Rotational thromboelastometry (ROTEM) allows to monitor hemostatic treatment with the possibility of specific treatment intervention.
Aims: To highlight the importance of ROTEM.
Methods: We are reporting the perioperative management in 21- and 22-year old patients with von Willebrand disease type 3 (vWD) and severe hemophilia A (HemA) during ankle arthrodesis. Standard laboratory tests and ROTEM-guided therapy were used.
Results: To the patient with vWD there was preoperatively administered 30 IU/kg FVIII/vWF concentrate. The basal levels before substitution were: FVIII: 34%, vWF activity: 16%, vWF antigen: 30%, aPTT: 32.4s and clotting time (CT) of INTEM: 178s. After the surgery these levels were normalized: FVIII: 103.5%, vWF activity: 84%, VWF antigen: 138% and aPTT: 29.2s. During the first 48h after the surgery, the patient was administered 20IU/kg FVIII/vWF every 8h. The patient was discharged home after 96h with aPTT 26.8s and control CT of INTEM 148s.
To the patient with severe HemA there was preoperatively administered rFVIII 50 IU/kg. After the surgery, FVIII level was 78% and CT of INTEM was 255s. During the first 24h after the surgery, the patient was administered rFVIII 30 IU/kg every 8h. The FVIII level was maintained 89±1% and CT of INTEM 200±5s. On the 1st postoperative day, the rFVIII dose was changed to 35 IU/kg every 12h. FVIII level achieved 76-66% and CT of INTEM 174-181s. Based on the CT of INTEM and FVIII level, the rFVIII dose was reduced to 20 IU/kg every 12h. On the 7th postoperative day, the patient was discharged home with a FVIII level 52% and CT of INTEM 82s. Bleeding symptoms were not observed in both patients.
Conclusion(s): Combination of standard coagulation testing and ROTEM represents adequate method for designing perioperative management in patients with inherited bleeding disorders.
To cite this abstract in AMA style:Sterankova M, Simurda T, Zolkova J, Brunclikova M, Stasko J, Skornova I, Cibula Z, Nečas L. Value of rotational thrombelastometry in perioperative management of patients with inherited coagulopathy during surgery [abstract]. https://abstracts.isth.org/abstract/value-of-rotational-thrombelastometry-in-perioperative-management-of-patients-with-inherited-coagulopathy-during-surgery/. Accessed September 29, 2023.
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