Abstract Number: LPB0077
Meeting: ISTH 2021 Congress
Background: Venous malformations (VMs) contain dilated venous vessels and are complicated with the activation of coagulation. Localized intravascular coagulation (LIC) may cause microthrombi in the lesion, therefore causing an increase in the dimension of the lesion and causing pain. Moreover, the localized activation of coagulation can lead to a consumptive coagulopathy characterized by increased D-dimers and decreased fibrinogen and platelets leading to bleeding tendency. Low molecular weight heparin (LMWH) therapy is an effective therapy in restoring normal coagulation in VM patients, however, the lifelong requirement of subcutaneous use is not easy in small children. Here we report, the first use of rivaroxaban, a direct oral anticoagulant drug in 6 pediatric patients with coagulopathy of venous malformations.
Aims: The aim of the study was to evaluate the clinical responses and coagulation laboratory tests of the patients under rivaroxaban therapy.
Methods: Between March 2020-December 2020, 6 pediatric patients aged between 2-12 years with VM and LIC have been transited to rivaroxaban therapy as they had been using LMWH for 3-6 months. Approval for off-label use of rivaroxaban from the Ministry of Health and informed consent was obtained for each patient. Platelet counts, fibrinogen and D-dimer levels, and clinical findings of the patients were retrospectively analyzed before and after rivaroxaban therapy. Clinical response was defined as a decrease in pain and strain over the VM.
Results: 
Figure 1. Lesions of patients with vascular malformations at the beginning of rivaroxaban therapy
The mean age of the patients was 6.33 (2-11.5 ages). The mean d-dimer levels significantly decreased after a median time of 3 months of (2-8 months) rivaroxaban therapy. The clinical, laboratory findings of the patients and responses to therapy are given in Table 1. The lesions before therapy were given in Figure 1. All of them showed clinical responses and no bleeding regarding VM.
| Patients | Age | Sex | Region of venous malformation | Previous medications | Rivaroxaban dose | D-dimer-before therapy | D-dimer- after therapy |
Clinical response | Side effects | ||
| 1 | 2 | M | Left cheek | Sirolimus, LMWH | 2×5 mg | 1.14 | 0.24 | + | None | ||
| 2 | 4 | F | Right toe, left lower leg | Sirolimus,propranolol,LMWH | 2×5 mg | 2.08 | 0.22 | + | None | ||
| 3 | 5 | M | Left lower and higher leg, left thigh | Propranolol, LMWH | 2×5 mg | 0.96 | 0.21 | + | None | ||
| 4 | 6.5 | M | Left cheek | Sirolimus,propranolol,LMWH | 2×5 mg | 3.2 | 0.19 | + | None | ||
| 5 |
9 | M | Left cervical region | LMWH | 2×5 mg | 3.65 | 0.22 | + | None | ||
| 6 | 11.5 | M | Left lower leg, left foot | Sirolimus, propranolol, LMWH | 2×10 mg | 3.91 | 0.31 | + | None |
Conclusions: Rivaroxaban therapy can be safely used in LIC of venous malformations in children.
To cite this abstract in AMA style:
Belen Apak FB, Nabili F, Sarialioglu F. Management of Localized Intravascular Coagulopathy with Rivaroxaban in Children with Venous Malformations: Report of 6 Cases [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/management-of-localized-intravascular-coagulopathy-with-rivaroxaban-in-children-with-venous-malformations-report-of-6-cases/. Accessed December 11, 2023.« Back to ISTH 2021 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/management-of-localized-intravascular-coagulopathy-with-rivaroxaban-in-children-with-venous-malformations-report-of-6-cases/