Treatment of Deep Vein Thrombosis in Patients with Neurofibromatosis
Initial Management
- The American College of Chest Physicians recommends beginning with parenteral anticoagulation while transitioning to oral anticoagulation for patients with deep vein thrombosis (DVT) 3
- For acute DVT without contraindications, the American College of Chest Physicians suggests initiating a direct-acting oral anticoagulant (DOAC) over vitamin K antagonist (VKA) therapy 2
- If using VKA therapy, the American College of Chest Physicians recommends starting it early and continuing parenteral anticoagulation for at least 5 days and until the INR is ≥2.0 4
- Home treatment is recommended for patients with adequate home circumstances, according to the American College of Chest Physicians 1
Duration of Treatment
- For provoked DVT, the American College of Chest Physicians recommends treating with anticoagulation for 3 months, regardless of whether the patient has neurofibromatosis 5, 2, 1
- For a first unprovoked proximal DVT, the American College of Chest Physicians suggests extended anticoagulation if bleeding risk is low or moderate 6
- For a second unprovoked VTE, the American College of Chest Physicians recommends extended anticoagulant therapy for those with low bleeding risk, and suggests extended therapy for those with moderate bleeding risk 7
Choice of Anticoagulant
- For patients without cancer, the American College of Chest Physicians suggests DOAC over VKA therapy 2
- For patients with cancer, the American College of Chest Physicians suggests low-molecular-weight heparin (LMWH) over VKA, DOAC, or other oral agents 2
- If using VKA, the American College of Chest Physicians recommends maintaining a therapeutic INR range of 2.0-3.0 (target INR of 2.5) 7
Additional Interventions
- The American College of Chest Physicians recommends IVC filters only for patients who have contraindications to anticoagulation 1
- Routine use of an IVC filter in addition to anticoagulation is not recommended by the American College of Chest Physicians 1
- The American College of Chest Physicians suggests anticoagulation alone over interventional therapy for most patients 1
- For patients who develop post-thrombotic syndrome (PTS), the American College of Chest Physicians suggests a trial of compression bandages or sleeves to reduce symptoms 1
Monitoring and Follow-up
- The American College of Chest Physicians recommends reassessing the need for continued anticoagulation at periodic intervals (e.g., annually) in patients receiving extended therapy 2