Fletcher Factor Deficiency and Myocardial Infarction

Currimbhoy, Z; Vlnciguerra, Vincent; Palakavongs, P; Kuslansky, Philip; Degnan, Thomas J.

doi:10.1093/ajcp/65.6.970

Cited by 40 publications

(23 citation statements)

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“…This finding was also shown in the study of Currimbhoy et a1 [3]. This may suggest that the congenital defect is not only autosomal recessive but in some families may have incomplete penetrance in females.…”

Section: Discussionsupporting

confidence: 75%

“…There are numerous reports of congenital defects of blood coagulation factors associated with arterial and venous thromboses, and this case of Fletcher factor deficiency may be a further example of the association of a thrombotic process occurring in a patient with a congenital coagulation defect. Myocardial infarction has been documented in factor VIII, factor IX, and factor XII deficiencies as well as Fletcher factor deficiency [3,. Severe factor XI1 deficiency has also been described in association with systemic arterial embolization, presumably from atrial mural thrombi [20].…”

Section: Discussionmentioning

confidence: 99%

“…In 1965 Hathaway et al reported four siblings from a consanguineous union with Fletcher factor deficiency [ 11. Since that time additional instances have been documented [2,3]. Eight of these ten cases have been described in Negro families, and males and females have been equally represented [4].…”

Section: Introductionmentioning

confidence: 99%

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Multiple cerebral thrombosis in fletcher factor (prekallikrein) deficiency: A case report

et al. 1985

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Despite markedly prolonged activated partial thromboplastin times (APTT) patients with Fletcher factor (prekallikrein) deficiency do not clinically bleed. However, there is one reported case of myocardial infarction associated with prekallikrein deficiency. These clinical observations suggest that the contact mechanism has a minor role in normal in vivo hemostatic function. We report a further two cases of prekallikrein deficiency in Caucasian siblings. The propositus presented with multiple cerebral thrombosis. Cautious anticoagulation resulted in massive cerebral hemorrhage and death of this patient. The sibling was investigated in an attempt to establish a possible defective fibrinolytic pathway in vivo. New sensitive tests for fibrinolysis were used. These included radioimmunoassay of fibrin degradation product "D," B beta 15-42, and in vitro 125I-labelled fibrin lysis assays. The plasma half-life of prekallikrein in this patient was calculated to be 58 hr. Family studies of heterozygotes were also performed. Prekallikrein deficiency is found not to be important in normal in vivo fibrinolysis, which possibly operates via tissue or vessel wall fibrinolytic activator release.

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Section: Discussionsupporting

confidence: 75%

Section: Discussionmentioning

confidence: 99%

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Multiple cerebral thrombosis in fletcher factor (prekallikrein) deficiency: A case report

et al. 1985

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“…They belonged to 56 separate kindreds [10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47]. A thrombotic event was reported in 9 of these patients belonging to 6 different families (table 1) [5,16,22,23,24,28]. …”

Section: Resultsmentioning

confidence: 99%

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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“…In 1973, Wuepper [22] was able to show that Fletcher factor is identical with prekallikrein (PKK). Fletcher trait seems to be a rare disorder since only a small number of cases have been re ported so far [ 1,2,5,10,13,17]. We report the case ofa 59-year-old patient with hyperthyroid ism (Graves' disease) in whom diagnosis of Fletcher factor deficiency was established dur ing routine preoperative laboratory screening.…”

Section: Introductionmentioning

confidence: 99%

CRM<sup>+</sup> Severe Fletcher Factor Deficiency Associated with Graves’ Disease

Kyrle¹,

Niessner²,

Deutsch³

et al. 1984

Pathophysiol Haemos Thromb

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A 59-year-old male patient with Graves’ disease and severe hereditary Fletcher factor deficiency is described. PKK clotting activity as well as the activity by a chromogenic substrate method (Chromozym PK®) was less then 0.01 U/ml. In contrast to functional tests, the immunological assay (Laurell method) showed a PKK antigen concentration of 0.25 U/ml, indicating the presence of an abnormal nonfunctional PKK molecule (CRM⁺ variant). An inhibitor was excluded since the patient plasma did not inactivate partially purified PKK. Investigation of 11 family members revealed a reduction of the PKK clotting activity in 9 relatives of the patient. Since Graves’ disease is considered an autoimmune disease, our case represents an example of an association of a severe hereditary deficiency of a contact factor and an autoimmune disease.

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Fletcher Factor Deficiency and Myocardial Infarction

Cited by 40 publications

References 0 publications

Multiple cerebral thrombosis in fletcher factor (prekallikrein) deficiency: A case report

Multiple cerebral thrombosis in fletcher factor (prekallikrein) deficiency: A case report

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

CRM<sup>+</sup> Severe Fletcher Factor Deficiency Associated with Graves’ Disease

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