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Paeds Vivashaematology-oncology-and-transfusion

Paeds Vivas · haematology-oncology-and-transfusion

Venous thromboembolism, pulmonary embolism and thrombophilia in children — branching viva

Branching viva on paediatric venous thromboembolism, pulmonary embolism and thrombophilia: the provoked and central-venous-catheter-associated epidemiology, the Virchow triad pathophysiology modified by developmental haemostasis, the diagnosis with compression ultrasound and CT pulmonary angiography, the age-stratified low molecular weight heparin dosing and anti-factor Xa monitoring, the rivaroxaban option from the EINSTEIN-Jr trials, the selective approach to hereditary thrombophilia testing, and the care of neonates, critically ill children and the transitioning adolescent.

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Prompt
Paediatric ward: a six-year-old boy with acute lymphoblastic leukaemia on induction chemotherapy, a tunnelled central venous catheter, and a swollen warm right leg, with a compression ultrasound confirming a femoral deep vein thrombosis.

Examiner opening (Examiner)

You are the general paediatric registrar on the ward. A six-year-old boy with acute lymphoblastic leukaemia, three months into induction chemotherapy with a tunnelled central venous catheter, presents with a swollen warm right leg over 48 hours. A compression ultrasound confirms a right femoral deep vein thrombosis. Talk me through your assessment and management plan. [3]

Exemplar opening (Candidate)

This boy has a provoked deep vein thrombosis. The central venous catheter is the dominant provoking factor in paediatric thrombosis, accounting for around half of all events, and the leukaemia itself and the induction chemotherapy — particularly asparaginase, which depletes antithrombin — create a hypercoagulable state. The combination is classic. My management is to start therapeutic anticoagulation with low molecular weight heparin, enoxaparin 1 mg per kg subcutaneously every 12 hours, check the anti-factor Xa level after the third or fourth dose with a target of 0.5 to 1.0 IU per mL for twice-daily dosing, treat for 6 weeks to 3 months, and remove the central line once it is no longer needed and the clot has stabilised. I will involve the paediatric haematology team early. [1] [7]

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References8ShowHide
  1. [1]Monagle P, Chan AKC, Goldenberg NA, et al. Antithrombotic therapy in neonates and children: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest, 2012.PMID 22315277
  2. [2]Monagle P, Cuello CA, Augustine C, et al. American Society of Hematology 2018 Guidelines for management of venous thromboembolism: treatment of pediatric venous thromboembolism. Blood Adv, 2018.PMID 30482766
  3. [3]Raffini L, Huang YS, Witmer C, et al. Dramatic increase in venous thromboembolism in children's hospitals in the United States from 2001 to 2007. Pediatrics, 2009.PMID 19736261
  4. [4]Male C, Lensing AWA, Palumbo JS, et al. Rivaroxaban compared with standard anticoagulants for the treatment of acute venous thromboembolism in children: a randomised, controlled, phase 3 trial. Lancet Haematol, 2020.PMID 31699660
  5. [6]Thom K, Lensing AWA, Nurmeev I, et al. Safety and efficacy of anticoagulant therapy in pediatric catheter-related venous thrombosis (EINSTEIN-Jr CVC-VTE). Blood Adv, 2020.PMID 33002131
  6. [7]Law C, Raffini L A guide to the use of anticoagulant drugs in children. Paediatr Drugs, 2015.PMID 25711916
  7. [8]van Ommen CH, Nowak-Göttl U Inherited Thrombophilia in Pediatric Venous Thromboembolic Disease: Why and Who to Test. Front Pediatr, 2017.PMID 28352625
  8. [11]Monagle P Diagnosis and management of deep venous thrombosis and pulmonary embolism in neonates and children. Semin Thromb Hemost, 2012.PMID 23034828
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