- Case report
- Open Access
- Open Peer Review
Acute myocardial infarction in a patient with hypofibrinogenemia: a case report
© Mghaieth et al; licensee BioMed Central Ltd. 2011
- Received: 25 September 2011
- Accepted: 19 December 2011
- Published: 19 December 2011
Congenital fibrinogen deficiency is a rare coagulation disorder usually responsible for hemorrhagic diathesis. However, it can be associated with thrombosis and there have been limited reports of arterial thrombotic complications in these patients.
A 42-year-old Tunisian man with congenital hypofibrinogenemia and no cardiovascular risk factors presented with new onset prolonged angina pectoris. An electrocardiogram showed features of inferior acute myocardial infarction. His troponin levels had reached 17 ng/L. Laboratory findings confirmed hypofibrinogenemia and ruled out thrombophilia. Echocardiography was not useful in providing diagnostic elements but did show preserved left ventricular function. Coronary angiography was not performed and our patient did not receive any anticoagulant treatment due to the major risk of bleeding. Magnetic resonance imaging confirmed myocardial necrosis. Our patient was managed with aspirin, a beta-blocker, an angiotensin-converting enzyme inhibitor and statin medication. The treatment was well tolerated and no ischemic recurrence was detected.
Although coronary thrombosis is a rare event in patients with fibrinogen deficiency, this condition is of major interest in view of the difficulties observed in managing these patients.
- Acute Myocardial Infarction
- Coronary Compute Tomography Angiography
- Coronary Thrombosis
- Thrombotic Complication
- Ischemic Recurrence
Hypo- and afibrinogenemia are rare congenital coagulation disorders with an estimated incidence of one in 500,000 births . They are usually responsible for an increased bleeding tendency. Although uncommon, some cases of venous and arterial thrombotic events associated with fibrinogen deficiency have been reported in the literature [1–9]. The mechanism of this apparently paradoxical thrombotic tendency, which has long remained problematic, has now been partially clarified .
We report the case of a 42-year-old Tunisian man, born out of a consanguineous marriage, who had no cardiovascular risk factors and was followed-up for hypofibrinogenemia diagnosed three years previously due to bleeding after dental care. He had no known family history of fibrinogen deficiency. He was admitted for an acute typical anginal pain which occurred at rest and continued for several hours.
Cases of arterial spontaneous thrombotic complications in patients with fibrinogen deficiency reported in the literature
Age (years), sex
Iliac artery thrombosis and toe necrosis
Ischemic necrosis of the first left toe
Bilateral distal ischemia of fingers and toes
Ischemic necrosis of the right foot due to popliteal artery thrombosis
Ischemic lesions of the feet secondary to an occlusive lesion of the hypogastric artery and highly stenotic lesions of the iliac arteries (two episodes at three-year interval). After surgical bypass, asymptomatic occlusion of the bypass occurred at Day three.
Ischemic stroke in a context of aortic marastic endocarditis
Ischemia of the fifth toe then of the fourth toe
Inferior acute myocardial infarction
Toe ischemia by bilateral thrombosis of the anterior tibial arteries
The increased risk of thrombosis in cases of fibrinogen deficiency could be explained by a rise in the level of circulating thrombin which is no longer inactivated by fibrin (previously known as antithrombin I factor) [3, 10]. The thrombin, playing the role of platelet activator, favors the excretion of Von Willebrand factor from platelet alpha-granules. This later enhances platelet aggregation by binding to glycoprotein IIb/IIIa, leading to the formation of loose large thrombi . Concerning the pathophysiologic mechanism, antiaggregant treatments can be effective in preventing the genesis of thrombotic events. Our patient received only aspirin; the patient described by Kumar et al.  received dual antiplatelet therapy (aspirin and clopidogrel). Neither of these two patients had an ischemic recurrence or a hemorrhagic complication.
Arterial thromboses are at present recognized as possible, even if rare, manifestations of fibrinogen deficiencies. In addition to the particularity of their pathology, these thromboses raise diagnostic and therapeutic problems due to the precarious stability of the hemostasis balance in these patients.
Written informed consent was obtained from the patient for publication of this manuscript and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
The authors are grateful to Richard Medeiros, Rouen University Hospital Medical Editor, for editing the manuscript.
- Lak M, Keihani M, Elahi F, Peyvandi F, Mannucci PM: Bleeding and thrombosis in 55 patients with inherited afibrinogenaemia. Br J Haematol. 1999, 107: 204-206. 10.1046/j.1365-2141.1999.01681.x.View ArticlePubMedGoogle Scholar
- Kumar N, Padma Kumar R, Ramesh B, Garg N: Afibrinogenaemia: a rare cause of young myocardial infarct. Singapore Med J. 2008, 49: 104-106.Google Scholar
- Dupuy E, Soria C, Molho P, Zini JM, Rosenstingl S, Laurian C, Bruneval P, Tobelem G: Embolized ischemic lesions of toes in an afibrinogenemic patient: possible relevance to in vivo circulating thrombin. Thromb Res. 2001, 102: 211-219. 10.1016/S0049-3848(01)00247-X.View ArticlePubMedGoogle Scholar
- Chun R, Poon MC, Haigh J, Seal D, Donahue B, Royston D: Cardiac surgery in congenital afibrinogenemia with thrombo-occlusive disease. J Cardiothorac Vasc Anesth. 2005, 19: 109-117. 10.1053/j.jvca.2004.11.021.View ArticlePubMedGoogle Scholar
- Castaman G, Lunardi M, Rigo L, Mastroeni V, Bonoldi E, Rodeghiero F: Severe spontaneous arterial thrombotic manifestations in patients with inherited hypo- and afibrinogenemia. Haemophilia. 2009, 15: 533-537. 10.1111/j.1365-2516.2009.01939.x.View ArticlePubMedGoogle Scholar
- Molho-Sabatier P, Soria C, Legrand Ch: Fibrinogen-independent platelet aggregation responsible of arterial thrombosis in an afibrinogemic patient. Thromb Haemost. 1991, 65: 850-Google Scholar
- Hanano M, Takahashi H, Itoh M, Shibata A: Coexistence of congenital afibrinogenemia and protein C deficiency in a patient. Am J Hematol. 1992, 41: 57-60. 10.1002/ajh.2830410111.View ArticlePubMedGoogle Scholar
- Chafa O, Chellali T, Sternberg C, Reghis A, Hamladji RM, Fischer AM: Severe hypofibrinogenemia associated with bilateral ischemic necrosis of toes and fingers. Blood Coagul Fibrinolysis. 1995, 6: 549-552. 10.1097/00001721-199509000-00008.View ArticlePubMedGoogle Scholar
- Gouider E, Ben Salah N, Aissaoui L, Jeddi R, Zorgati M, El Borji W, Hafsia R: Afibrinogenemia and gangrene. A case report. JIM Sfax. 2007, 13: 44-45.Google Scholar
- Chevalier Y, Dargaud Y, Argaud L, Ninet J, Jouannou E, Negrier C: Successive bleeding and thrombotic complications in a patient with afibrinogenemia: a case report. Thromb Res. 2011, 128: 296-298. 10.1016/j.thromres.2011.03.012.View ArticlePubMedGoogle Scholar
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