Semin Thromb Hemost 2017; 43(07): 653-671
DOI: 10.1055/s-0037-1605568
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Managing and Supporting Surgery in Patients with Bleeding Disorders

Jennifer Curnow
1   Department of Clinical and Laboratory Hematology, Institute of Clinical Pathology and Medical Research, Westmead Hospital, Sydney Centers for Thrombosis and Haemostasis, Westmead, New South Wales, Australia
› Author Affiliations
Further Information

Publication History

Publication Date:
13 September 2017 (online)

Abstract

Patients with inherited bleeding disorders are likely to require surgery at various times throughout life. In some cases, this may be major orthopaedic surgery required due to their underlying condition, for example, hemophilic arthropathy. For inherited disorders with significant bleeding risk, surgery should take place in a hemophilia treatment center with the availability of hematologic, anesthetic, surgical, nursing, scientific laboratory, and allied health expertise. Preoperative assessment, planning, and communication between team members are crucial. Specific replacement therapy is usually required, as may other adjunctive therapies. Venous thromboembolism (VTE) risk assessment is individualized, taking account of other underlying risks in the patient and risks associated with the procedure, including the replacement agent itself, and use of mechanical prophylaxis may be the safest approach in many cases. Pain management is also modified to take account of risks associated with the bleeding disorder, and spinal or epidural analgesia is restricted compared with patients who do not have hemostatic defects. In patients with acquired bleeding disorders, the preoperative assessment includes decisions regarding how best to optimize management of the underlying condition prior to proceeding with surgery. If this is achieved, specific replacement therapy may not be required. If complete remission of the acquired bleeding disorder is achieved preoperatively, then VTE prophylaxis and pain management options will not be limited by the bleeding disorder. The perioperative management of both hereditary and acquired bleeding disorders is discussed and contrasted.

 
  • References

  • 1 Kazmi RS, Boyce S, Lwaleed BA. Homeostasis of hemostasis: the role of endothelium. Semin Thromb Hemost 2015; 41 (06) 549-555
  • 2 Jaffe EA, Hoyer LW, Nachman RL. Synthesis of von Willebrand factor by cultured human endothelial cells. Proc Natl Acad Sci U S A 1974; 71 (05) 1906-1909
  • 3 Sporn LA, Chavin SI, Marder VJ, Wagner DD. Biosynthesis of von Willebrand protein by human megakaryocytes. J Clin Invest 1985; 76 (03) 1102-1106
  • 4 Sadler JE, Budde U, Eikenboom JC. , et al; Working Party on von Willebrand Disease Classification. Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor. J Thromb Haemost 2006; 4 (10) 2103-2114
  • 5 Yee A, Kretz CA. Von Willebrand factor: form for function. Semin Thromb Hemost 2014; 40 (01) 17-27
  • 6 Peyvandi F, Garagiola I, Baronciani L. Role of von Willebrand factor in the haemostasis. Blood Transfus 2011; 9 (Suppl. 02) s3-s8
  • 7 Heemskerk JW, Mattheij NJ, Cosemans JM. Platelet-based coagulation: different populations, different functions. J Thromb Haemost 2013; 11 (01) 2-16
  • 8 Sørensen JV. Levels of fibrinolytic activators and inhibitors in plasma after severe trauma. Blood Coagul Fibrinolysis 1994; 5 (01) 43-49
  • 9 Borgstrom S, Gelin LE, Zederfeldt B. The formation of vein thrombi following tissue injury: an experimental study in rabbits. Acta Chir Scand Suppl 1959; 247 (Suppl. 247) 1-36
  • 10 Kluft C, Verheijen JH, Jie AF. , et al. The postoperative fibrinolytic shutdown: a rapidly reverting acute phase pattern for the fast-acting inhibitor of tissue-type plasminogen activator after trauma. Scand J Clin Lab Invest 1985; 45 (07) 605-610
  • 11 Kambayashi J, Sakon M, Yokota M, Shiba E, Kawasaki T, Mori T. Activation of coagulation and fibrinolysis during surgery, analyzed by molecular markers. Thromb Res 1990; 60 (02) 157-167
  • 12 Myers DD, Hawley AE, Farris DM. , et al. P-selectin and leukocyte microparticles are associated with venous thrombogenesis. J Vasc Surg 2003; 38 (05) 1075-1089
  • 13 Martini WZ. Coagulopathy by hypothermia and acidosis: mechanisms of thrombin generation and fibrinogen availability. J Trauma 2009; 67 (01) 202-208 , discussion 208–209
  • 14 Hamer JD, Malone PC, Silver IA. The PO2 in venous valve pockets: its possible bearing on thrombogenesis. Br J Surg 1981; 68 (03) 166-170
  • 15 Comerota AJ, Stewart GJ, Alburger PD, Smalley K, White JV. Operative venodilation: a previously unsuspected factor in the cause of postoperative deep vein thrombosis. Surgery 1989; 106 (02) 301-308 , discussion 308–309
  • 16 Avorn J, Patel M, Levin R, Winkelmayer WC. Hetastarch and bleeding complications after coronary artery surgery. Chest 2003; 124 (04) 1437-1442
  • 17 Aglietti P, Baldini A, Vena LM, Abbate R, Fedi S, Falciani M. Effect of tourniquet use on activation of coagulation in total knee replacement. Clin Orthop Relat Res 2000; (371) 169-177
  • 18 Levy JH, Sniecinski RM. Prohemostatic treatment in cardiac surgery. Semin Thromb Hemost 2012; 38 (03) 237-243
  • 19 Ranucci M. Hemostatic and thrombotic issues in cardiac surgery. Semin Thromb Hemost 2015; 41 (01) 84-90
  • 20 Collins R, Scrimgeour A, Yusuf S, Peto R. Reduction in fatal pulmonary embolism and venous thrombosis by perioperative administration of subcutaneous heparin. Overview of results of randomized trials in general, orthopedic, and urologic surgery. N Engl J Med 1988; 318 (18) 1162-1173
  • 21 Bevan DH. Cardiac bypass haemostasis: putting blood through the mill. Br J Haematol 1999; 104 (02) 208-219
  • 22 Woodman RC, Harker LA. Bleeding complications associated with cardiopulmonary bypass. Blood 1990; 76 (09) 1680-1697
  • 23 Kitchens CS. Occult hemophilia. Johns Hopkins Med J 1980; 146 (06) 255-259
  • 24 Zumberg MS, Waples JM, Kao KJ, Lottenberg R. Management of a patient with a mechanical aortic valve and antibodies to both thrombin and factor V after repeat exposure to fibrin sealant. Am J Hematol 2000; 64 (01) 59-63
  • 25 Favaloro EJ, Posen J, Ramakrishna R. , et al. Factor V inhibitors: rare or not so uncommon? A multi-laboratory investigation. Blood Coagul Fibrinolysis 2004; 15 (08) 637-647
  • 26 White II GC, Rosendaal F, Aledort LM, Lusher JM, Rothschild C, Ingerslev J. ; Factor VIII and Factor IX Subcommittee. Definitions in hemophilia. Recommendation of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 2001; 85 (03) 560
  • 27 Australian Haemophilia Centre Directors' Organisation (AHCDO). Guidelines for the Management of Haemophilia in Australia. 2016
  • 28 Srivastava A, Brewer AK, Mauser-Bunschoten EP. , et al; Treatment Guidelines Working Group on Behalf of The World Federation Of Hemophilia. Guidelines for the management of hemophilia. Haemophilia 2013; 19 (01) e1-e47
  • 29 Shander A, Javidroozi M. The approach to patients with bleeding disorders who do not accept blood-derived products. Semin Thromb Hemost 2013; 39 (02) 182-190
  • 30 Curnow J, Pasalic L, Favaloro EJ. Why do patients bleed?. Surg J 2016; 2 (01) e29-e43
  • 31 Escobar M, Maahs J, Hellman E. , et al. Multidisciplinary management of patients with haemophilia with inhibitors undergoing surgery in the United States: perspectives and best practices derived from experienced treatment centres. Haemophilia 2012; 18 (06) 971-981
  • 32 Kempton CL, Soucie JM, Miller CH. , et al. In non-severe hemophilia A the risk of inhibitor after intensive factor treatment is greater in older patients: a case-control study. J Thromb Haemost 2010; 8 (10) 2224-2231
  • 33 Eckhardt CL, van der Bom JG, van der Naald M, Peters M, Kamphuisen PW, Fijnvandraat K. Surgery and inhibitor development in hemophilia A: a systematic review. J Thromb Haemost 2011; 9 (10) 1948-1958
  • 34 Gouw SC, van den Berg HM, Oldenburg J. , et al. F8 gene mutation type and inhibitor development in patients with severe hemophilia A: systematic review and meta-analysis. Blood 2012; 119 (12) 2922-2934
  • 35 Castaman G, Fijnvandraat K. Molecular and clinical predictors of inhibitor risk and its prevention and treatment in mild hemophilia A. Blood 2014; 124 (15) 2333-2336
  • 36 Batorova A, Martinowitz U. Intermittent injections vs. continuous infusion of factor VIII in haemophilia patients undergoing major surgery. Br J Haematol 2000; 110 (03) 715-720
  • 37 Othman M. Rare bleeding disorders: genetic, laboratory, clinical, and molecular aspects. Preface. Semin Thromb Hemost 2013; 39 (06) 575-578
  • 38 Peyvandi F, Menegatti M, Palla R. Rare bleeding disorders: worldwide efforts for classification, diagnosis, and management. Semin Thromb Hemost 2013; 39 (06) 579-584
  • 39 Peyvandi F, Palla R, Menegatti M. , et al; European Network of Rare Bleeding Disorders Group. Coagulation factor activity and clinical bleeding severity in rare bleeding disorders: results from the European Network of Rare Bleeding Disorders. J Thromb Haemost 2012; 10 (04) 615-621
  • 40 Palla R, Peyvandi F, Shapiro AD. Rare bleeding disorders: diagnosis and treatment. Blood 2015; 125 (13) 2052-2061
  • 41 Salomon O, Steinberg DM, Seligshon U. Variable bleeding manifestations characterize different types of surgery in patients with severe factor XI deficiency enabling parsimonious use of replacement therapy. Haemophilia 2006; 12 (05) 490-493
  • 42 Nichols WL, Hultin MB, James AH. , et al. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA). Haemophilia 2008; 14 (02) 171-232
  • 43 Favaloro EJ. Von Willebrand disease: local diagnosis and management of a globally distributed bleeding disorder. Semin Thromb Hemost 2011; 37 (05) 440-455
  • 44 Favaloro EJ. Diagnosing von Willebrand disease: a short history of laboratory milestones and innovations, plus current status, challenges, and solutions. Semin Thromb Hemost 2014; 40 (05) 551-570
  • 45 Ng C, Motto DG, Di Paola J. Diagnostic approach to von Willebrand disease. Blood 2015; 125 (13) 2029-2037
  • 46 Lillicrap D. von Willebrand disease: advances in pathogenetic understanding, diagnosis, and therapy. ASH Education Program Book; 2013: 254-260
  • 47 Favaloro EJ, Bodó I, Israels SJ, Brown SA. von Willebrand disease and platelet disorders. Haemophilia 2014; 20 (Suppl. 04) 59-64
  • 48 Flood VH. Perils, problems, and progress in laboratory diagnosis of von Willebrand disease. Semin Thromb Hemost 2014; 40 (01) 41-48
  • 49 Curnow J, Pasalic L, Favaloro EJ. Treatment of von Willebrand Disease. Semin Thromb Hemost 2016; 42 (02) 133-146
  • 50 Othman M. Platelet-type von Willebrand disease: a rare, often misdiagnosed and underdiagnosed bleeding disorder. Semin Thromb Hemost 2011; 37 (05) 464-469
  • 51 Othman M, Kaur H, Emsley J. Platelet-type von Willebrand disease: new insights into the molecular pathophysiology of a unique platelet defect. Semin Thromb Hemost 2013; 39 (06) 663-673
  • 52 Favaloro EJ, Pasalic L, Curnow J. Monitoring therapy during treatment of von Willebrand disease. Semin Thromb Hemost 2017; 43 (03) 338-354
  • 53 Favaloro EJ, Pasalic L, Curnow J. Type 2M and 2M von Willebrand disease: similar but different. Semin Thromb Hemost 2016; 42 (05) 483-497
  • 54 Althaus K, Greinacher A. MYH9-related platelet disorders. Semin Thromb Hemost 2009; 35 (02) 189-203
  • 55 Selleng K, Lubenow LE, Greinacher A, Warkentin TE. Perioperative management of MYH9 hereditary macrothrombocytopenia (Fechtner syndrome). Eur J Haematol 2007; 79 (03) 263-268
  • 56 Gresele P, Bury L, Falcinelli E. Inherited platelet function disorders: algorithms for phenotypic and genetic investigation. Semin Thromb Hemost 2016; 42 (03) 292-305
  • 57 Lowe GC, Lordkipanidzé M, Watson SP. ; UK GAPP study group. Utility of the ISTH bleeding assessment tool in predicting platelet defects in participants with suspected inherited platelet function disorders. J Thromb Haemost 2013; 11 (09) 1663-1668
  • 58 Alamelu J, Liesner R. Modern management of severe platelet function disorders. Br J Haematol 2010; 149 (06) 813-823
  • 59 Blanchette VS, Key NS, Ljung LR, Manco-Johnson MJ, van den Berg HM, Srivastava A. ; Subcommittee on Factor VIII, Factor IX and Rare Coagulation Disorders of the Scientific and Standardization Committee of the International Society on Thrombosis and Hemostasis. Definitions in hemophilia: communication from the SSC of the ISTH. J Thromb Haemost 2014; 12 (11) 1935-1939
  • 60 Hermans C, Altisent C, Batorova A. , et al; European Haemophilia Therapy Standardisation Board. Replacement therapy for invasive procedures in patients with haemophilia: literature review, European survey and recommendations. Haemophilia 2009; 15 (03) 639-658
  • 61 Rickard KA. Guidelines for therapy and optimal dosages of coagulation factors for treatment of bleeding and surgery in haemophilia. Haemophilia 1995; 1 (Suppl. 01) 8-13
  • 62 Björkman S, Berntorp E. Pharmacokinetics of coagulation factors: clinical relevance for patients with haemophilia. Clin Pharmacokinet 2001; 40 (11) 815-832
  • 63 Poon M-C, Lillicrap D, Hensman C, Card R, Scully M-F. Recombinant factor IX recovery and inhibitor safety: a Canadian post-licensure surveillance study. Thromb Haemost 2002; 87 (03) 431-435
  • 64 Martinowitz U, Schulman S, Gitel S, Horozowski H, Heim M, Varon D. Adjusted dose continuous infusion of factor VIII in patients with haemophilia A. Br J Haematol 1992; 82 (04) 729-734
  • 65 Boban A, Henrard S, Lambert C, Hermans C. Continuous infusion of factor VIII concentrates in obese patients with severe haemophilia A: is weight-based dose-adjustment required?. Haemophilia 2016; 22 (01) e62-e64
  • 66 Graham A, Jaworski K. Pharmacokinetic analysis of anti-hemophilic factor in the obese patient. Haemophilia 2014; 20 (02) 226-229
  • 67 Mahdi AJ, Obaji SG, Collins PW. Role of enhanced half-life factor VIII and IX in the treatment of haemophilia. Br J Haematol 2015; 169 (06) 768-776
  • 68 Kempton CL, White II GC. How we treat a hemophilia A patient with a factor VIII inhibitor. Blood 2009; 113 (01) 11-17
  • 69 Roberts HR, Monroe DM, White GC. The use of recombinant factor VIIa in the treatment of bleeding disorders. Blood 2004; 104 (13) 3858-3864
  • 70 Turecek PL, Varadi K, Gritsch H. , et al. Factor Xa and prothrombin: mechanism of action of FEIBA. Vox Sang 1999; 77 (Suppl. 01) 72-79
  • 71 Dargaud Y, Lambert T, Trossaert M. New advances in the therapeutic and laboratory management of patients with haemophilia and inhibitors. Haemophilia 2008; 14 (Suppl. 04) 20-27
  • 72 Favaloro EJ, Franchini M, Lippi G. Biological therapies for von Willebrand disease. Expert Opin Biol Ther 2012; 12 (05) 551-564
  • 73 Laffan MA, Lester W, O'Donnell JS. , et al. The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology. Br J Haematol 2014; 167 (04) 453-465
  • 74 de Wee EM, Leebeek FW, Eikenboom JC. Diagnosis and management of von Willebrand disease in The Netherlands. Semin Thromb Hemost 2011; 37 (05) 480-487
  • 75 Mannucci PM, Franchini M. The use of plasma-derived concentrates. In: Federici AB, Lee CA, Berntorp EE, Lillicrap D, Montgomery RR. , eds. Von Willebrand Disease. 2nd ed. West Sussex, UK: Blackwell Publishing Ltd; 2010
  • 76 Dunkley S, Baker RI, Pidcock M. , et al. Clinical efficacy and safety of the factor VIII/von Willebrand factor concentrate BIOSTATE in patients with von Willebrand's disease: a prospective multi-centre study. Haemophilia 2010; 16 (04) 615-624
  • 77 Mannucci PM, Kempton C, Millar C. , et al; rhVWF Ad Hoc Study Group. Pharmacokinetics and safety of a novel recombinant human von Willebrand factor manufactured with a plasma-free method: a prospective clinical trial. Blood 2013; 122 (05) 648-657
  • 78 Gill JC, Castaman G, Windyga J. , et al. Hemostatic efficacy, safety, and pharmacokinetics of a recombinant von Willebrand factor in severe von Willebrand disease. Blood 2015; 126 (17) 2038-2046
  • 79 Batlle J, López-Fernández MF, Fraga EL, Trillo AR, Pérez-Rodríguez MA. Von Willebrand factor/factor VIII concentrates in the treatment of von Willebrand disease. Blood Coagul Fibrinolysis 2009; 20 (02) 89-100
  • 80 Estcourt LJ, Birchall J, Allard S. , et al; British Committee for Standards in Haematology. Guidelines for the use of platelet transfusions. Br J Haematol 2017; 176 (03) 365-394
  • 81 Nurden P, Nurden AT. Congenital disorders associated with platelet dysfunctions. Thromb Haemost 2008; 99 (02) 253-263
  • 82 Poon MC, d'Oiron R, Hann I. , et al. Use of recombinant factor VIIa (NovoSeven) in patients with Glanzmann thrombasthenia. Semin Hematol 2001; 38 (04) (Suppl. 12) 21-25
  • 83 Dargaud Y, Bordet JC, Trzeciak MC, Vinciguerra C, Negrier C. A case of Glanzmann's thrombasthenia successfully treated with recombinant factor VIIa during a surgical procedure: observations on the monitoring and the mechanism of action of this drug. Haematologica 2006; 91 (6, Suppl): ECR20
  • 84 McKay H, Derome F, Haq MA. , et al. Bleeding risks associated with inheritance of the Quebec platelet disorder. Blood 2004; 104 (01) 159-165
  • 85 Rodeghiero F. Von Willebrand disease: pathogenesis and management. Thromb Res 2013; 131 (Suppl. 01) S47-S50
  • 86 Tosetto A, Castaman G. How I treat type 2 variant forms of von Willebrand disease. Blood 2015; 125 (06) 907-914
  • 87 Federici AB, Mazurier C, Berntorp E. , et al. Biologic response to desmopressin in patients with severe type 1 and type 2 von Willebrand disease: results of a multicenter European study. Blood 2004; 103 (06) 2032-2038
  • 88 Favaloro EJ, Kershaw G, Bukuya M, Hertzberg M, Koutts J. Laboratory diagnosis of von Willebrand disorder (vWD) and monitoring of DDAVP therapy: efficacy of the PFA-100 and vWF:CBA as combined diagnostic strategies. Haemophilia 2001; 7 (02) 180-189
  • 89 Favaloro EJ. Rethinking the diagnosis of von Willebrand disease. Thromb Res 2011; 127 (Suppl. 02) S17-S21
  • 90 Nieuwenhuis HK, Sixma JJ. 1-Desamino-8-D-arginine vasopressin (desmopressin) shortens the bleeding time in storage pool deficiency. Ann Intern Med 1988; 108 (01) 65-67
  • 91 Lethagen S, Nilsson IM. DDAVP-induced enhancement of platelet retention: its dependence on platelet-von Willebrand factor and the platelet receptor GP IIb/IIIa. Eur J Haematol 1992; 49 (01) 7-13
  • 92 Coppola A, Di Minno G. Desmopressin in inherited disorders of platelet function. Haemophilia 2008; 14 (Suppl. 01) 31-39
  • 93 Cattaneo M, Zighetti ML, Lombardi R, Mannucci PM. Role of ADP in platelet aggregation at high shear: studies in a patient with congenital defect of platelet responses to ADP. Br J Haematol 1994; 88 (04) 826-829
  • 94 Rybo G. Tranexamic acid therapy: effective treatment in heavy menstrual bleeding: a clinical update on safety. Therapeutic Adv 1991; 4: 1-8
  • 95 Teitel JM, Carcao M, Lillicrap D. , et al. Orthopaedic surgery in haemophilia patients with inhibitors: a practical guide to haemostatic, surgical and rehabilitative care. Haemophilia 2009; 15 (01) 227-239
  • 96 Muntean W, Zenz W, Edlinger G, Beitzke A. Severe bleeding due to factor V inhibitor after repeated operations using fibrin sealant containing bovine thrombin. Thromb Haemost 1997; 77 (06) 1223
  • 97 Ozelo MC. Surgery in patients with hemophilia: is thromboprophylaxis mandatory?. Thromb Res 2012; 130 (Suppl. 01) S23-S26
  • 98 Journeycake JM, Quinn CT, Miller KL, Zajac JL, Buchanan GR. Catheter-related deep venous thrombosis in children with hemophilia. Blood 2001; 98 (06) 1727-1731
  • 99 Hermans C. Venous thromboembolic disease in patients with haemophilia. Thromb Res 2012; 130 (Suppl. 01) S50-S52
  • 100 Butcher JH, Pasi KJ. Fatal postoperative pulmonary embolism in mild haemophilia. Haemophilia 2006; 12 (02) 179-182
  • 101 Geerts WH, Bergqvist D, Pineo GF. , et al. Prevention of venous thromboembolism: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th edition). Chest 2008; 133: 3815-5435
  • 102 Zakarija A, Aledort L. How we treat: venous thromboembolism prevention in haemophilia patients undergoing major orthopaedic surgery. Haemophilia 2009; 15 (06) 1308-1310
  • 103 Pradhan SM, Key NS, Boggio L, Pruthi R. Venous thrombosis prophylaxis in haemophilics undergoing major orthopaedic surgery: a survey of haemophilia treatment centres. Haemophilia 2009; 15 (06) 1337-1338
  • 104 Girolami A, Scandellari R, Zanon E, Sartori R, Girolami B. Non-catheter associated venous thrombosis in hemophilia A and B. A critical review of all reported cases. J Thromb Thrombolysis 2006; 21 (03) 279-284
  • 105 Franchini M. Thrombotic complications in patients with hereditary bleeding disorders. Thromb Haemost 2004; 92 (02) 298-304
  • 106 Kraaijenhagen RA, in't Anker PS, Koopman MM. , et al. High plasma concentration of factor VIIIc is a major risk factor for venous thromboembolism. Thromb Haemost 2000; 83 (01) 5-9
  • 107 Kyrle PA, Minar E, Hirschl M. , et al. High plasma levels of factor VIII and the risk of recurrent venous thromboembolism. N Engl J Med 2000; 343 (07) 457-462
  • 108 Makris M, Colvin B, Gupta V, Shields ML, Smith MP. Venous thrombosis following the use of intermediate purity FVIII concentrate to treat patients with von Willebrand's disease. Thromb Haemost 2002; 88 (03) 387-388
  • 109 Coppola A, Franchini M, Makris M, Santagostino E, Di Minno G, Mannucci PM. Thrombotic adverse events to coagulation factor concentrates for treatment of patients with haemophilia and von Willebrand disease: a systematic review of prospective studies. Haemophilia 2012; 18 (03) e173-e187
  • 110 Franchini M, Targher G, Montagnana M, Lippi G. Antithrombotic prophylaxis in patients with von Willebrand disease undergoing major surgery: when is it necessary?. J Thromb Thrombolysis 2009; 28 (02) 215-219
  • 111 Girolami A, Tezza F, Scandellari R, Vettore S, Girolami B. Associated prothrombotic conditions are probably responsible for the occurrence of thrombosis in almost all patients with congenital FVII deficiency. Critical review of the literature. J Thromb Thrombolysis 2010; 30 (02) 172-178
  • 112 Ruiz-Sáez A. Thrombosis in rare bleeding disorders. Hematology 2012; 17 (Suppl. 01) S156-S158
  • 113 Rattray B, Nugent DJ, Young G. Celecoxib in the treatment of haemophilic synovitis, target joints, and pain in adults and children with haemophilia. Haemophilia 2006; 12 (05) 514-517
  • 114 Tsoukas C, Eyster ME, Shingo S. , et al. Evaluation of the efficacy and safety of etoricoxib in the treatment of hemophilic arthropathy. Blood 2006; 107 (05) 1785-1790
  • 115 Eyster ME, Asaad SM, Gold BD, Cohn SE, Goedert JJ. ; Second Multicenter Hemophilia Study Group. Upper gastrointestinal bleeding in haemophiliacs: incidence and relation to use of non-steroidal anti-inflammatory drugs. Haemophilia 2007; 13 (03) 279-286
  • 116 Vanarase MY, Pandit H, Kimstra YW, Dodd CAF, Popat MT. Pain relief after knee replacement in patients with a bleeding disorder. Haemophilia 2007; 13 (04) 395-397
  • 117 Lee CA, Chi C, Pavord SR. , et al; UK Haemophilia Centre Doctors' Organization. The obstetric and gynaecological management of women with inherited bleeding disorders--review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors' Organization. Haemophilia 2006; 12 (04) 301-336
  • 118 Demers C, Derzko C, David M, Douglas J. ; Society of Obstetricians and Gynecologists of Canada. Gynaecological and obstetric management of women with inherited bleeding disorders. J Obstet Gynaecol Can 2005; 27 (07) 707-732
  • 119 Chi C, Lee CA, England A, Hingorani J, Paintsil J, Kadir RA. Obstetric analgesia and anaesthesia in women with inherited bleeding disorders. Thromb Haemost 2009; 101 (06) 1104-1111
  • 120 Stasi R. How to approach thrombocytopenia. Hematology (Am Soc Hematol Educ Program) 2012; 2012: 191-197
  • 121 Aster RH. Pooling of platelets in the spleen: role in the pathogenesis of “hypersplenic” thrombocytopenia. J Clin Invest 1966; 45 (05) 645-657
  • 122 Kim J, Huh R, Jaffer AK. Perioperative approach to anticoagulants and hematologic disorders. Anesthesiol Clin 2016; 34 (01) 101-125
  • 123 Rodeghiero F, Stasi R, Gernsheimer T. , et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood 2009; 113 (11) 2386-2393
  • 124 Neunert C, Noroozi N, Norman G. , et al. Severe bleeding events in adults and children with primary immune thrombocytopenia: a systematic review. J Thromb Haemost 2015; 13 (03) 457-464
  • 125 Godeau B, Chevret S, Varet B. , et al; French ATIP Study Group. Intravenous immunoglobulin or high-dose methylprednisolone, with or without oral prednisone, for adults with untreated severe autoimmune thrombocytopenic purpura: a randomised, multicentre trial. Lancet 2002; 359 (9300): 23-29
  • 126 Mithoowani S, Gregory-Miller K, Goy J. , et al. High-dose dexamethasone compared with prednisone for previously untreated primary immune thrombocytopenia: a systematic review and meta-analysis. Lancet Haematol 2016; 3 (10) e489-e496
  • 127 Arnold DM, Dentali F, Crowther MA. , et al. Systematic review: efficacy and safety of rituximab for adults with idiopathic thrombocytopenic purpura. Ann Intern Med 2007; 146 (01) 25-33
  • 128 Provan D, Stasi R, Newland AC. , et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood 2010; 115 (02) 168-186
  • 129 Zaja F, Barcellini W, Cantoni S. , et al. Thrombopoietin receptor agonists for preparing adult patients with immune thrombocytopenia to splenectomy: results of a retrospective, observational GIMEMA study. Am J Hematol 2016; 91 (05) E293-E295
  • 130 Salama A, Rieke M, Kiesewetter H, von Depka M. Experiences with recombinant FVIIa in the emergency treatment of patients with autoimmune thrombocytopenia: a review of the literature. Ann Hematol 2009; 88 (01) 11-15
  • 131 Kojouri K, Vesely SK, Terrell DR, George JN. Splenectomy for adult patients with idiopathic thrombocytopenic purpura: a systematic review to assess long-term platelet count responses, prediction of response, and surgical complications. Blood 2004; 104 (09) 2623-2634
  • 132 Boyle S, White RH, Brunson A, Wun T. Splenectomy and the incidence of venous thromboembolism and sepsis in patients with immune thrombocytopenia. Blood 2013; 121 (23) 4782-4790
  • 133 Franchini M, Mannucci PM. Acquired haemophilia A: a 2013 update. Thromb Haemost 2013; 110 (06) 1114-1120
  • 134 Delgado J, Jimenez-Yuste V, Hernandez-Navarro F, Villar A. Acquired haemophilia: review and meta-analysis focused on therapy and prognostic factors. Br J Haematol 2003; 121 (01) 21-35
  • 135 Franchini M, Lippi G. Acquired factor VIII inhibitors. Blood 2008; 112 (02) 250-255 [Erratum in Blood 2009;113(21):5368. Dosage error in article text.]
  • 136 Sallah S. Treatment of acquired haemophilia with factor eight inhibitor bypassing activity. Haemophilia 2004; 10 (02) 169-173
  • 137 Hay CR, Negrier C, Ludlam CA. The treatment of bleeding in acquired haemophilia with recombinant factor VIIa: a multicentre study. Thromb Haemost 1997; 78 (06) 1463-1467
  • 138 Gomperts E. Recombinant B domain deleted porcine factor VIII for the treatment of bleeding episodes in adults with acquired hemophilia A. Expert Rev Hematol 2015; 8 (04) 427-432
  • 139 Navarrete MA, van der Meer FJ, Damiani G, Diaz A, Eikenboom J. The use of rituximab therapy in patients with acquired factor V inhibitors. Am J Hematol 2012; 87 (08) 826-827
  • 140 Boehlen F, Casini A, Chizzolini C. , et al. Acquired factor XIII deficiency: a therapeutic challenge. Thromb Haemost 2013; 109 (03) 479-487
  • 141 Federici AB, Budde U, Castaman G, Rand JH, Tiede A. Current diagnostic and therapeutic approaches to patients with acquired von Willebrand syndrome: a 2013 update. Semin Thromb Hemost 2013; 39 (02) 191-201
  • 142 Budde U, Bergmann F, Michiels JJ. Acquired von Willebrand syndrome: experience from 2 years in a single laboratory compared with data from the literature and an international registry. Semin Thromb Hemost 2002; 28 (02) 227-238
  • 143 Federici AB, Rand JH, Bucciarelli P. , et al; Subcommittee on von Willebrand Factor. Acquired von Willebrand syndrome: data from an international registry. Thromb Haemost 2000; 84 (02) 345-349
  • 144 Friederich PW, Wever PC, Briët E, Doorenbos CJ, Levi M. Successful treatment with recombinant factor VIIa of therapy-resistant severe bleeding in a patient with acquired von Willebrand disease. Am J Hematol 2001; 66 (04) 292-294
  • 145 Sadler JE. Aortic stenosis, von Willebrand factor, and bleeding. N Engl J Med 2003; 349 (04) 323-325
  • 146 Lippi G, Pasalic L, Favaloro EJ. Detection of mild inherited disorders of blood coagulation: current options and personal recommendations. Expert Rev Hematol 2015; 8 (04) 527-542
  • 147 Mumford AD, Ackroyd S, Alikhan R. , et al; BCSH Committee. Guideline for the diagnosis and management of the rare coagulation disorders: a United Kingdom Haemophilia Centre Doctors' Organization guideline on behalf of the British Committee for Standards in Haematology. Br J Haematol 2014; 167 (03) 304-326
  • 148 Srivastava A, Brewer AK, Mauser-Bunschoten EP. , et al. WFH Guidelines for the Management of Hemophilia. 2nd ed. Available at: http://www.wfh.org/en/resources/wfh-treatment-guidelines? Accessed February 11, 2015
  • 149 Rydz N, James PD. Approach to the diagnosis and management of common bleeding disorders. Semin Thromb Hemost 2012; 38 (07) 711-719