Von Willebrand Factor Antigen and Factor XI Activity Levels As Predictors of Bleeding Tendency in Israeli Patients with Von Willebrand's Disease

Brenner, Benjamin; Stemberg, Tamar; Laor, Arieh; Tavori, S.; Tatarsky, Ilana; Lanir, Naomi

doi:10.1177/107602969500100402

Cited by 5 publications

(4 citation statements)

References 20 publications

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“…Most of the bleeding in partial factor XI deficiency could be explained by the presence of von Willebrand disease [36]. Conversely, in another study, the bleeding tendency in individuals with von Willebrand disease was predicted by the factor XI:C level and von Willebrand factor antigen level using logistic regression analysis [18]. Using similar methods in patients with factor XI deficiency, bleeding was predicted with a specificity of 85%.…”

Section: Clinical Picture and Inheritancementioning

confidence: 99%

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Factor XI deficiency and its management

Bolton‐Maggs

2000

Haemophilia

108

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Factor XI deficiency has a more variable bleeding tendency than haemophilia A or B. Individuals with severe deficiency have only a mild bleeding tendency, which is typically provoked by surgery, but the risk of bleeding is not restricted to individuals with severe deficiency. The bleeding tendency varies between individuals with similar factor XI levels, and sometimes the bleeding tendency of an individual may vary. The reasons for this are not fully understood, although in cases of severe deficiency there is some correlation between phenotype and genotype. Factor XI is activated by thrombin. The role of factor XI in physiological processes has become clearer since this fact was discovered, and the discovery has contributed to a revised model of blood coagulation. Factor XI deficiency occurs in all racial groups, but is particularly common in Ashkenazi Jews. The factor XI gene is 23 kilobases long. Two mutations are responsible for most factor XI deficiency in the Ashkenazi population, but a number of other mutations have now been reported in other racial groups. Individuals with factor XI deficiency may need specific therapy for surgery, accidents, and dental extractions. Several therapies are available which include fresh frozen plasma, factor XI concentrates, fibrin glue, antifibrinolytic drugs, and desmopressin. Each has advantages and risks to be considered. Factor XI concentrate may be indicated for procedures with a significant risk of bleeding especially in younger patients with severe deficiency, but its use in older patients has been associated with thrombotic phenomena. If fresh frozen plasma is to be used it is preferable to obtain one of the virally inactivated products. Fibrin glue is a useful treatment which deserves further study.

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Section: Clinical Picture and Inheritancementioning

confidence: 99%

“…Researchers noted the lack of absolute relationship between bleeding history and factor XI [11]. Further studies have confirmed that between 20 and 50% of individuals with partial deficiency bleed excessively [14–19]. Figure 2 shows data from studies in the United Kingdom.…”

Section: Clinical Picture and Inheritancementioning

confidence: 99%

Factor XI deficiency and its management

Bolton‐Maggs

2000

Haemophilia

108

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“…1, Table 1, and the ROC analysis suggest that low FVIII levels may be a modest risk factor for bleeding in some patients with FXI levels ≤ 60%. While this could be owing to a reduced production of thrombin at sites of injury, a low FVIII level could also be a surrogate for a concurrently low level of von Willebrand factor (VWF), which has also been reported to be a risk for bleeding in some [1,6,13], but not all [2] previously reported studies on FXI deficiency. VWF (antigen or ristocetin cofactor) values were obtained on only 47 of the 72 subjects discussed above.…”

Section: Factor (F)viii Values In Two Groups Of Factor (F)xi‐deficienmentioning

confidence: 99%

“…Therefore, the possibility that a low FVIII value in some patients from the larger group may be a surrogate for impaired VWF‐mediated platelet function can not be ruled out. The findings in these patients referred for workup of a bleeding disorder, as well as kindreds from large families with FXI deficiency, indicate that a low level of FVIII [1], or VWF [1,6,13], may be a modest risk factor for bleeding in patients with FXI deficiency. They also suggest the prophylactic or therapeutic use of 1‐8‐deamino‐ d ‐arginine vasopressin (DDAVP) to control bleeding in selected patients [1].…”

Section: Factor (F)viii Values In Two Groups Of Factor (F)xi‐deficienmentioning

confidence: 99%

Low factor VIII levels are a risk factor for bleeding in patients with factor XI deficiency

Weiss

2005

Journal of Thrombosis and Haemostasis

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To cite this article: Weiss HJ. Low factor VIII levels are a risk factor for bleeding in patients with factor XI deficiency. J Thromb Haemost 2005; 3: 181-2.Studies on kindreds from large families with factor (F)XI deficiency strongly suggest that low plasma levels of FXI, although probably important [1][2][3], do not necessarily predict the presence or magnitude of a bleeding tendency [1,3,4]. It has been suggested that patients who bleed may have additional hemostatic defects, such as decreased levels of platelet FXI coagulant activity [5]. The results of some [1], but not all [2,6], studies have suggested that low plasma levels of factor (F)VIII may be an additional risk factor for bleeding. To examine whether the FVIII level influences the bleeding tendency in patients deficient in FXI, I used information I had obtained on 564 patients (excluding 47 with thrombocytopenia) who were referred to me either for an evaluation of a bleeding problem, an abnormal laboratory value, or membership in a family with a bleeding disorder [7].FVIII and FXI values in these patients had been determined by standard one-stage methods based on the activated partial thromboplastin time [8]. Substrates for these assays were plasmas from patients severely deficient in these factors, and individual values were calculated from a curve constructed from the pooled plasmas of 20-25 normal hospital personnel. A FXI level £ 60% was considered abnormal, consistent with previous reports [1][2][3][4]6,9], and was observed in 81 of 217 subjects in whom FXI values had been obtained. The magnitude of the bleeding tendency in each patient was estimated by a bleeding score (t) calculated by adding the weighted values for each of six bleeding symptoms: bleeding after cuts, surgical procedures, or dental procedures, easy bruising, epistaxis, and gastrointestinal bleeding [8]. To examine whether the level of FVIII influenced the bleeding tendency in these patients, the correlation between the FVIII level and the bleeding score was determined and found to be highly significant (r ¼ ) 0.32, P ¼ 0.01). However, this analysis included nine patients, four with hemophilia and five with various types of von Willebrand disease, whose FVIII levels were < 30%, values that, by themselves, might be associated with impaired hemostasis [10,11]. Therefore, the correlation between the bleeding score (t) and FVIII levels was assessed after excluding these patients. The scatter plot on the patients with FVIII values ‡ 30% is shown in Fig. 1. Despite the considerable scatter of the data, a modest, but significant, correlation of the bleeding score with the FVIII level was again observed (r ¼ ) 0.31, P ¼ 0.01, n ¼ 72).To examine further whether low FVIII values increased the risk for bleeding in the 72 patients with FXI deficiency described above, they were divided into two clinically distinct groups (Table 1). Group I included all patients in whom bleeding symptoms were either absent (t ¼ 0) or were characterized as easy bruising or a single episode of bleeding in anot...

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10 Factor XI deficiency

Bolton‐Maggs¹

1996

Baillière's Clinical Haematology

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Von Willebrand Factor Antigen and Factor XI Activity Levels As Predictors of Bleeding Tendency in Israeli Patients with Von Willebrand's Disease

Cited by 5 publications

References 20 publications

Factor XI deficiency and its management

Factor XI deficiency and its management

Low factor VIII levels are a risk factor for bleeding in patients with factor XI deficiency

10 Factor XI deficiency

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