Myocardial Infarction, Other Arterial Thrombosis and Invasive Coronary Procedures, in Hemaophilia B: A Critical Evaluation of Reported Cases

Girolami, Antonio; Randi, Maria Luigia; Ruzzon, Elisabetta; Zanon, Ezio; Girolami, Bruno

doi:10.1007/s11239-005-2227-3

Cited by 41 publications

(64 citation statements)

References 22 publications

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“…44 However, longitudinal studies in the Netherlands of patients with factor VIII or factor IX deficiency demonstrated a lower incidence of myocardial infarction than in controls, and an 80% reduction in mortality from coronary artery disease, suggesting factor VIII or IX deficiency may inhibit formation of thrombi at sites of plaque rupture. 45,46 Although 2 recent reviews of case reports concluded that myocardial infarction may be more prevalent than previously suspected in hemophilia A or B, 47,48 a significant number of cases were associated with factor replacement or the use of thrombogenic prothrombin complex concentrates. Since the 1960's, a role for elevated factor VIII levels in coronary and cerebrovascular disease has been addressed by several large studies (reviewed in reference 36), with conflicting results.…”

Section: Intrinsic Pathway Proteins and Thromboembolic Disease In Humansmentioning

confidence: 99%

Intrinsic Pathway of Coagulation and Arterial Thrombosis

Gailani

Renné

2007

ATVB

247

172

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Abstract-Formation of a fibrin clot is mediated by a group of tightly regulated plasma proteases and cofactors. While this system is essential for minimizing blood loss from an injured blood vessel (hemostasis), it also contributes to pathologic fibrin formation and platelet activation that may occlude vessels (thrombosis). Many antithrombotic drugs target key elements of the plasma coagulation mechanism such as thrombin and factor Xa, based on the premise that plasma elements contributing to thrombosis are primarily those involved in hemostasis. Recent studies with genetically altered mice raise questions about this paradigm. Deficiencies of the intrinsic pathway proteases factor XII and factor XI are not associated with abnormal hemostasis in mice, but impair formation of occlusive thrombi in arterial injury models, indicating that pathways not essential for hemostasis participate in arterial thrombosis. If factor XII or factor XI make similar contributions to thrombosis in humans, these proteases could be ideal targets for drugs to treat or prevent thromboembolic disease with minimal risk of therapy-associated bleeding. (Arterioscler Thromb Vasc

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Section: Intrinsic Pathway Proteins and Thromboembolic Disease In Humansmentioning

confidence: 99%

Intrinsic Pathway of Coagulation and Arterial Thrombosis

Gailani

Renné

2007

ATVB

247

172

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“…This clearly indicated that the bleeding conditions did not fully protect from thrombosis. These papers have dealt mainly with hemophilia and von Willebrand disease patients but even rare bleeding disorders have been occasionally involved [1,[5][6][7][8].…”

Section: Introductionmentioning

confidence: 99%

Associated prothrombotic conditions are probably responsible for the occurrence of thrombosis in almost all patients with congenital FVII deficiency. Critical review of the literature

Girolami

Tezza

Scandellari

et al. 2010

J Thromb Thrombolysis

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The occasional occurrence of thrombosis in patients with congenital bleeding disorders has received considerable attention during the past decade. Myocardial infarction, ischemic strokes and venous thromboembolism have been reported in hemophilia A or B patients, in von Willebrand disease and, also, in rare coagulation disorders, especially in factor VII (FVII) deficiency. To explain the relatively high frequency of thrombotic phenomena, mainly venous, seen in the last condition, it was speculated that a special form or variant of FVII deficiency could exist. The presence of associated prothrombotic risk factors has been occasionally reported to be present in these patients but the matter has never been duly evaluated and emphasized. The purpose of the present paper was to evaluate if the clinical setting in which thrombosis appeared in these patients could explain the occurrence of the thrombosis. All reported cases of thrombosis seen in patients with FVII deficiency have been analyzed and the presence of associated risk factors recorded. Out of a population of 33 documented cases, the presence of prothrombotic risk factors was reported in 30 instances. In two of the remaining cases, no mention is made about associated risk factors. In the last case they were explicitly excluded. The critical evaluation of the literature suggests that the occurrence of thrombosis in FVII deficiency may be due to common prothrombotic risk factors. As a consequence it may be only stated that FVII deficiency does not protect from thrombosis.

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“…Several thrombotic complications have been reported in patients with congenital bleeding disorders with the exception of prothrombin or FX deficiency [1,2,3,4,5]. These arterial or venous thromboses occur most often during or immediately after substitution with coagulation factors [1,2].…”

Section: Introductionmentioning

confidence: 99%

“…These arterial or venous thromboses occur most often during or immediately after substitution with coagulation factors [1,2]. Occasionally they appear spontaneously.…”

Section: Introductionmentioning

confidence: 99%

“…Occasionally they appear spontaneously. Due to the higher prevalence of hemophilia A or B and von Willebrand’s disease, more patients with these disorders are affected by thrombosis [1,2,4]. Among the rare coagulation disorders, FVII deficiency seems to be most frequently associated with thrombosis and this also appears to be due to the higher prevalence of this condition in comparison with other rare congenital bleeding disorders [6].…”

Section: Introductionmentioning

confidence: 99%

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Thrombotic Events in Patients with Congenital Prekallikrein Deficiency: A Critical Evaluation of All Reported Cases

et al. 2010

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The occurrence of thrombotic events in patients with congenital bleeding conditions has received considerable attention in recent years. The same is true for asymptomatic defects of factors of the contact phase of blood coagulation, mainly FXII. Anecdotal reports on thrombosis in patients with prekallikrein deficiency have occasionally been reported. These involved both arterial and venous thrombosis. The purpose of the present article is to analyze the stories and the clinical pictures of all 75 cases of prekallikrein deficiency published so far. Among these patients were 9 with thrombosis, 6 arterial (myocardial infarction and ischemic stroke) and 3 venous (deep vein thrombosis with or without pulmonary embolism). In 6 cases acquired thrombosis risk factors were present; in 2 cases no associated risk factors were present and in 1 case no information was supplied in this regard. One patient who presented both a stroke and a pulmonary embolism had a fatal outcome. The article clearly indicates that prekallikrein deficiency does not protect from thrombosis in spite of the severe in vitro coagulation defect.

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Myocardial Infarction, Other Arterial Thrombosis and Invasive Coronary Procedures, in Hemaophilia B: A Critical Evaluation of Reported Cases

Cited by 41 publications

References 22 publications

Intrinsic Pathway of Coagulation and Arterial Thrombosis

Intrinsic Pathway of Coagulation and Arterial Thrombosis

Associated prothrombotic conditions are probably responsible for the occurrence of thrombosis in almost all patients with congenital FVII deficiency. Critical review of the literature

Thrombotic Events in Patients with Congenital Prekallikrein Deficiency: A Critical Evaluation of All Reported Cases

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