Acute, symptomatic catheter‐associated DVT with mobile, intra‐cardiac thrombus in an infant with severe haemophilia A

Abstract

References 1 Franchini M, Favaloro EJ, Lippi G. Mild hemophilia A. J Thromb Haemost 2010; 8: 421-32. 2 Hay CR. Factor VIII inhibitors in mild and moderate-severity haemophilia A. Haemophilia 1998; 4: 558-63. 3 Mauser-Bunschoten EP, Den Uijl IE, Schutgens RE, Roosendaal G, Fischer K. Risk of inhibitor development in mild haemophilia A increases with age. Haemophilia 2012; 18: 263-7. 4 Hay CR, Ludlam CA, Colvin BT et al. Factor VIII inhibitors in mild and moderateseverity haemophilia A. Thromb Haemost 1998; 79: 762-6. 5 Eckhardt CL, Menke LA, Van Ommen CH et al. Intensive peri-operative use of factor VIII and the Arg593 > Cys mutation are risk factors for inhibitor development in mild/moderate hemophilia A. J Thromb Haemost 2009; 7: 930-7. 6 Cid AR, Casana P, Cabrera N, Haya S, Cortina V, Azmar JA. Inhibitor development in one patient and laboratory discrepancies in several families with both mild haemophilia and Arg531Cys mutation. Haemophilia 2007; 13: 206-8. 7 Shima M. Characterization of factor VIII inhibitors. Int J Hematol 2006; 83: 109-18. 8 Eckhardt CL, Mauser-Bunschoten EP, Peters M, Leebeek FW, van der Meer FJ, Fijnvandraat K. Inhibitor incidence after intensive FVIII replacement for surgery in mild and moderate haemophilia A: a prospective national study in the Netherlands. Br J Haematol 2012; 157: 747-52. 9 Shibata M, Shima M, Morichika S et al. An alloantibody recognizing the FVIII A1 domain in a patient with CRM reduced haemophilia A due to deletion of a large portion of the A1 domain DNA sequence. Thromb Haemost 2000; 84: 442-8. 10 d'Oiron R, Pipe SW, Jacquemin M. Mild/ moderate haemophilia A: new insights into molecular mechanisms and inhibitor development.The patient was a 6-month-old male with severe haemophilia A, who presented with fever, irritability, purulent drainage from his portacath needle access site, and bilateral upper extremity and chest oedema. The portacath was placed less than 8 weeks earlier and was his third central venous access device (CVAD) due to a history of recurrent CVAD-associated bacterial infections and severe peri-natal intracranial haemorrhage. At the time of this presentation, the patient was on a three times per week recombinant factor VIII prophylaxis regimen (32 units kg À1 per dose) administered at home by his mother, who had been taught sterile technique and to flush the device with saline and heparin (50 units) with each use. He was started on antibiotics and the portacath was removed on hospital day 1 after initiation of a perioperative factor replacement regimen. Blood cultures from the portacath ultimately grew methicillin-sensitive staphylococcus aureus and Enterobacter cloacae, with MSSA also growing from a peripheral blood culture. On hospital day 2, persistence and progression of the arm and chest oedema noted at presentation prompted an ultrasound that showed occlusive thrombi in the left internal jugular, innominate and subclavian veins. D-dimer at that time was elevated at 3.39 mcg mL À1 fibrinogen-equivalent units. A CT venogram was obtained for furth...