Abstract
The occurrence of thrombosis in patients with congenital bleeding disorders represents an exceptional event. Hemophilia A and hemophilia B patients have been showed to present both arterial and venous thrombosis (85 cases of arterial thrombosis and 34 cases of venous thrombosis). The great majority of arterial thrombosis are myocardial infarction or other acute coronary syndromes, whereas the majority of venous thrombosis are deep vein thrombosis and/or pulmonary embolisms. However there are discrepancies in the proportion of arterial and venous thrombosis seen in hemophilia A versus hemophilia B. The ratio of arterial versus venous thrombosis in hemophilia A is 3.72 whereas that for hemophilia B is 1.12. This indicates that arterial thrombosis is more frequent in hemophilia A as compared to hemophilia B and the opposite is true for venous thrombosis. The potential significance of this discrepancy is discussed.
Similar content being viewed by others
References
Dolan G (2010) The challenge of an ageing haemophilic population. Haemophilia 16(Suppl 5):11–16
Girolami A, Ruzzon E, Fabris F, Varvarikis C, Sartori R, Girolami B (2006) Myocardial infarction and other arterial occlusions in hemophilia a patients. A cardiological evaluation of all 42 cases reported in the literature. Acta Haematol 116:120–125
Girolami A, Randi ML, Ruzzon E, Zanon E, Girolami B (2005) Myocardial infarction, other arterial thrombosis and invasive coronary procedures, in hemaophilia B: a critical evaluation of reported cases. J Thromb Thrombolysis 20:43–46
Coppola A, De Simone C, Di Capua M, Tufano A, Cimino E, Conca P, Guida A, Morisco C, Di Minno G (2010) Acute coronary syndrome and severe haemophilia: an unusual association with challenging treatment. Thromb Haemost 103:1270–1272
Chavin SI, Siegel DM, Rocco TA Jr, Olson JP (1988) Acute myocardial infarction during treatment with an activated prothrombin complex concentrate in a patient with factor VIII deficiency and a factor VIII inhibitor. Am J Med 85:245–249
Dalldorf FG, Taylor RE, Blatt PM (1981) Arteriosclerosis in severe hemophilia. A postmortem study. Arch Pathol Lab Med 105:652–654
Girolami A, Scandellari R, Zanon E, Sartori R, Girolami B (2006) Non-catheter associated venous thrombosis in hemophilia A and B. A critical review of all reported cases (Review). J Thromb Thrombolysis 21:279–284
Bicer M, Yanar M, Tuydes O (2009) Spontaneous deep vein thrombosis in hemophilia A: a case report. Cases J 2:6390
Eleftheriadis N, Makris P (2010) Portal vein thrombosis in a patient with HCV cirrhosis and combined hemophilia A and thrombophilia V Leiden. Ther Clin Risk Manag 6:539–541
Eren A, Friedl R, Hannekum A, Gulbins H (2006) Cardiac surgery in a patient with haemophilia A. Thorac Cardiovasc Surg 54:212–214
Ergelen M, Osmonov D, Gunaydin ZY, Sahin O, Isik T, Uyarel H (2009) Acute inferior myocardial infarction in a patient with severe haemophilia A disease. Ann Hematol 88:711–712
Ferrario C, Renders F, Cairoli A, Vuffray A, Spertini O, Angelillo-Scherrer A (2007) Management of an acute coronary syndrome in a patient with severe haemophilia A. Haemophilia 13:763–765
Forman MB, Kopelman HA, Gleaton JH, Janco RL, Perry JM (1986) Percutaneous transluminal coronary angioplasty in hemophilia. Am Heart J 111:169–172
Guo Y, Zhang X, Huang D, Xi X, Liu Y, Zhu J (2010) Acute myocardial infarction in a Chinese patient with haemophilia A and risk factors for coronary artery disease: a case report with autopsy. Haemophilia 16:691–693
Helft G, Metzger JP, Samama MM, Rothschild C, Batisse JP, Vacheron A (1997) Coronary stenting in a hemophilic patient. Thromb Haemost 77(5):1044–1045
Hermans C, Hammer F, Lobet S, Lambert C (2010) Subclinical deep venous thrombosis observed in 10 % of hemophilic patients undergoing major orthopedic surgery. J Thromb Haemost 8:1138–1140
Karayalcin G, Goldberg B, Cherrick I, Kurer C, Bierman F, Lanzkowsky P (1993) Acute myocardial infarction complicating prothrombin complex concentrate therapy in an 8-year-old boy with hemophilia A and factor VIII inhibitor (Review). Am J Pediatr Hematol Oncol 15:416–419
Kashyap R, Sharma LM, Gupta S, Saxena R, Srivastava DN (2006) Deep vein thrombosis in a patient with severe haemophilia A. Haemophilia 12:87–89
Krolick MA (2005) Successful percutaneous coronary intervention in a patient with severe haemophilia A using bivalirudin as the sole procedural anticoagulant. Haemophilia 11:415–417
Lim MY, Pruthi RK (2011) Cardiovascular disease risk factors: prevalence and management in adult hemophilia patients. Blood Coagul Fibrinolysis 22:402–406
Kim DK, Kim DI, Kim MS, Lee EJ, Kim YB, Cho HJ, Han YC, Kim U, Seal SH, Yang TH, Kim DK, Kim DS (2010) Successful percutaneous coronary intervention for acute coronary syndrome in a patient with severe Hemophilia A. Korean Circ J 40:527–529
Pesaro AE, Gaz MV, Karbstein R, Perin M, Serrano CV, Damico E (2009) Coronary artery bypass surgery, angioplasty and long term anti-platelet treatment in a type B hemophilia patient. Clinics (Sao Paulo) 64:822–823
Petrillo G, Cirillo P, Leosco D, Maresca F, Piscione F, Chiariello M (2011) Percutaneous coronary intervention in a patient with acute non-ST-elevation myocardial infarction and haemophilia A: a ‘genous’ experience. Haemophilia 17(1):e245–e246
Quintero D, Biria M, Meyers DG (2008) Percutaneous coronary intervention in a patient with acute ST-elevation myocardial infarction and hemophilia A. J Invasive Cardiol 20:240–241
Robinson KL, Savoia H, Street AM (2000) Thrombotic complications in two patients receiving NovoSeven® (Abstract). Haemophilia 6:349
Scharfman WB, Rauch AE, Ferraris V, Burkart PT (1993) Treatment of a patient with factor IX deficiency (hemophilia B) with coronary bypass surgery. J Thorac Cardiovasc Surg 105:765–766
Smolka G, Kulach A, Dabek J, Szulc A, Gasior Z (2007) Percutaneous coronary intervention with stent implantation in haemophilic A patient with unstable angina. Haemophilia 13:428–431
Sullivan DW, Purdy LJ, Billingham M, Glader BE (1984) Fatal myocardial infarction following therapy with prothrombin complex concentrates in a young man with hemophilia A. Pediatrics 74:279–281
Zanon E, Gentilomo C, Laverda A, Suppiej A, Saggiorato G, Manara R, Simioni P (2008) Cerebral sinus venous thrombosis with parenchymal infarcts in a newborn with severe haemophilia A and subdural haematomas. Haemophilia 14:1117–1121
Sartori MT, Bilora F, Zanon E, Varvarikis C, Saggiorato G, Campagnolo E, Pagnan A, Cella G (2008) Endotelial disfunction in hemophilia patients. Haemophilia 14:1055–1062
Zwiers M, Lefrandt JD, Mulder DJ, Smit AJ, Gans RO, Vliegenthart R, Brands-Nijenhuis AV, Kluin-Nelemans JC, Meijer K (2012) Coronary artery calcification score and carotid intima–media thickness in patients with hemophilia. J Thromb Haemost 10:23–29
Goodnough L, Saito H, Ratnoff O (1983) Thrombosis and myocardial infarction in congenital clotting factor abnormalities and in chronic thrombocytopenia: a report of 21 patients and a review of 50 previously reported cases. Medicine 62:248–255
Girolami A, Molaro G, Falomo R (1975) Factor X Friuli coagulation disorder. The demise of the index patient. Acta Haematol 54:120–125
Vianello F, Belvini D, Dal Bello F, Tagariello G, Zanon E, Lombardi AM, Zerbinati P, Girolami A (2001) Mild bleeding diatesis in a boy with combined severe hemophilia B (C10400 > T) and heterozygous factor V Leiden. Haemophilia 7:511–514
Lim MY, Pruthi RK (2012) Outcomes of management of acute coronary syndrome in patients with congenital bleeding disorders: a single center experience and review of the literature. Thromb Res 130(3):316–322
Schutgens RE, Tuinenburg A, Roosendaal G, Guyomi SH, Mauser-Bunschoten EP (2009) Treatment of ischaemic heart disease in haemophilia patients: an institutional guideline. Haemophilia 15:952–958
Acknowledgments
This study was supported in part by the “Associazione per l’Emofilia ed altre Coagulopatie delle Tre Venezie”.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Girolami, A., Bertozzi, I., de Marinis, G.B. et al. Discrepant ratios of arterial versus venous thrombosis in hemophilia A as compared with hemophilia B. J Thromb Thrombolysis 37, 293–297 (2014). https://doi.org/10.1007/s11239-013-0943-7
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11239-013-0943-7