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PubMed2001

Current therapy for rare factor deficiencies.

Haemophilia A and B and von Willebrand disease account for 80-85% of all inherited bleeding disorders. The other 15% are represented by deficiencies of fibrinogen, prothrombin, or factors V, VII, X, XI, or XIII. In addition, acquired factor deficiencies are seen in a variety of conditions ranging from malignancies to autoimmune disorders. The spectrum of symptoms in these conditions varies from severe and life-threatening haemorrhage to a mild bleeding diathesis. The diagnosis depends on demonstration of decreased activity of one of the clotting factors. Due to the rarity of each of the individual factor deficiencies, purified factor concentrates are not as readily available as they are for haemophilia A and B. Treatment of rare clotting factor deficiencies consists of the most purified blood product available that contains the missing factor. Depending on which factor is deficient, either purified concentrates, prothrombin complex concentrates, cryoprecipitate, or fresh frozen plasma can be used. In addition, recombinant factor VIIa is available for treating factor VII deficient patients.

Blood Coagulation Factors↗
PubMed1993

Low molecular weight factor X activator from Cerastes vipera (Sahara sand viper) venom.

Fraction G from Cerastes vipera venom previously purified on Sephadex G100 was refractionated on DEAE-Sephadex A50 column. A factor X activator was obtained. It had a mol. wt of 12,500 and an isoelectric point of 4.4. It shortened the plasma recalcification time of normal plasma, and plasmas deficient in factors V, VII, VIII, IX, XI and XIII, while it had no effect on plasma deficient in factor X or factor II. It had a serine protease activity and a minimal plasmin activity. PMSF, leupeptin and iodoacetamide exerted a pronounced inhibitory effect on its serine protease activity. Polyantivenin could neutralize the coagulant activity of the activator.

Amino Acid Sequence↗
PubMed1977

Coagulopathies.

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Afibrinogenemia↗
PubMed2002

Increased risk for postoperative hemorrhage after intracranial surgery in patients with decreased factor XIII activity: implications of a prospective study.

BACKGROUND AND PURPOSE: The functional integrity of the hemostatic system is a prerequisite for the safe performance of neurosurgical procedures. To monitor the individual coagulation capacity of each patient, standard tests are effective to detect deficiencies involving the generation of fibrin. However, fibrin clot strength depends primarily on coagulation factor XIII, which cross-links fibrin monomers and enhances clot resistance against fibrinolysis. Therefore, factor XIII is functionally involved in both the hemostatic and fibrinolytic systems. The objective of this prospective study was to determine the incidence and clinical relevance of perioperative decreased factor XIII with respect to standard coagulation parameters and the occurrence of postoperative hematoma. METHODS: In 876 patients, 910 neurosurgical procedures were performed. Prothrombin time (PT), partial thromboplastin time (PTT), platelet count, fibrinogen, and factor XIII were tested in each patient preoperatively and postoperatively. RESULTS: Postoperative intracranial hematoma (defined as requiring surgical evacuation) occurred after 39 (4.3%) of 910 surgical procedures. Patients with postoperative hematoma had significantly lower factor XIII and fibrinogen levels preoperatively and postoperatively than patients without hematoma. In patients with postoperative hematoma, PT and platelets differed significantly only postoperatively, whereas PTT was different neither preoperatively nor postoperatively. Of the 39 patients with a postoperative hematoma, 13 (33.3%) had a postoperative factor XIII <60% compared with 61 (7%) of 867 patients without hematoma (P<0.01, Fisher's exact test). The relative risk of developing a postoperative hematoma is therefore increased 6.4-fold in patients with postoperative factor XIII <60%. The risk is increased 12-fold in patients who additionally have postoperative decreased fibrinogen levels (<1.5 g/L) and 9-fold in patients with platelet count <150x10(9)/L and factor XIII <60%. CONCLUSIONS: This is the first prospective study that demonstrates the association of decreased perioperative factor XIII with an increased risk of postoperative hematoma in neurosurgical patients. The risk is further increased in those patients with low factor XIII and additional abnormalities of fibrinogen, PT, platelets, and PTT. Factor XIII testing and specific replacement, as accepted for other clotting factors, may reduce the risk of postoperative hematoma.

Comorbidity↗
PubMedDate not supplied

Inhibitors to clotting factors.

Clot formation is the final result of interaction among multiple plasma proteins; after activation, it results in the conversion of fibrinogen to fibrin and cross-linking of fibrin by activated factor XIII, which stabilizes the formed clot. Deficiency or functional abnormality of the factors involved in these reactions causes bleeding disorders. Natural inhibitors of clotting factors include antithrombin III, protein S, and protein C. When activated, these proteins inactivate specific clotting factors, providing a regulatory mechanism that serves to control the coagulation response and limit the extension of the clot. Physiologic or natural inhibitors should not be confused with acquired inhibitors of coagulation factors, which are discussed in this review. Inhibitors to coagulation factors, also known as circulating anticoagulants, are antibodies that neutralize specific clotting proteins, thereby interfering with their normal function. Antibodies may be directed against isolated clotting factors, as is the case with factor VIII or IX inhibitors. On the other hand, the antiphospholipid antibodies are known to develop against multiple coagulation proteins. In contrast to patients with antibodies against isolated clotting factors, who commonly present with spontaneous bleeding, individuals with antiphospholipid antibodies may be asymptomatic or present with venous or arterial thrombosis. In this article I refer to inhibitors developing in patients with hemophilia A or other congenital factor deficiency as alloantibodies, and to spontaneous formation of antibodies in patients without prior history of hemorrhagic diathesis as autoantibodies. The antiphospholipid antibodies are discussed separately.

Adult↗
PubMed2001

Evaluation of a sensitive colorimetric FXIII incorporation assay. Effects of FXIII Val34Leu, plasma fibrinogen concentration and congenital FXIII deficiency.

There is an increasing interest in the role of coagulation factor XIII (FXIII) in cardio- and cerebrovascular diseases. It has recently been reported that a common G-->T point mutation in the A-subunit gene of FXIII, which codes for a valine (val) to leucine (leu) change (FXIIIVal34Leu), is protective against thrombotic diseases but seems to increase the risk of intracerebral bleeding. We developed a colorimetric incorporation assay for detection of FXIII activity based on incorporation of 5-(biotinamido) pentylamine (BAPA) into fibrin or fibrinogen. With this new assay, we studied the effects of FXIIIVal34Leu mutation, plasma fibrinogen concentration and congenital FXIII deficiency on FXIII activity. There are no data available about the ability of different FXIII assays to detect altered activity in FXIIIVal34Leu genotypes. We therefore compared our results determined by the incorporation method with a commonly used photometric method based on ammonia release after cross-linking of glycine-ethylester to a specific glutamine containing peptide substrate. We also determined FXIII A-subunit antigen (Ag) levels using enzyme-linked immunosorbent assay (ELISA) technique. The FXIIIVal34Leu genotype could not be detected either by the photometric method nor by the FXIII A-subunit ELISA. The incorporation assay showed an increased specific FXIII activity in subjects possessing the leu allele. The photometric assay and ELISA gave similar results independent from genotype. In patients with congenital FXIII deficiency before and after substitution, however, ELISA and the incorporation assay gave similar results, whereas the photometric assay showed consistently higher values. Our results show that the incorporation assay, not the photometric assay based on ammonia release, can be used for detection of elevated activity in subjects with FXIIIVal34Leu. Because of specificity and over a wide range sensitivity, the assay can also be used for determination of FXIII deficiency and monitoring of FXIII substitution therapy.

Amines↗
PubMed2004

[Congenital deficiencies of coagulation factors and acquired inhibitors leading to bleeding disorders].

This review focuses on symptoms, course and treatment of bleeding disorders due to hereditary coagulation factor deficiencies and acquired inhibitors, mentioning as well the pathophysiologic and molecular genetic aspects and diagnostic particularities. The review of haemophilia A and B deals with carrier problems, replacement therapy, additional haemostatic agents such as antifibrinolytics and desmopressin, the treatment of typical haemorrhages, haemophilia with inhibitors and future therapeutic options. Of the autosomal homozygous bleeding disorders such as von Willebrand disease type 3, afibrinogenaemia, factor XIII-, VII- and XI-deficiency each has its particularities influencing treatment strategies. The last chapter discusses acquired bleeding disorders such as acquired haemophilia A, von Willebrand disease, factor V deficiency and the hypoprothrombinaemia lupus anticoagulant syndrome, the different modes of inhibition, diagnostics and principles of treatment.

Blood Coagulation Disorders↗
PubMed2000

Horn fly (Diptera: Muscidae) saliva targets thrombin action in hemostasis.

The horn fly, Hematobia irritans (L.), is an important pest of livestock because the adult stage of both sexes are aggressive blood-feeders. Remarkably, even though horn fly adults feed recurrently on their hosts as ectoparasites, these flies lack the ADP-responsive antiplatelet aggregation and vasodilatory antihemostatic systems described for other blood-feeding Diptera. Horn fly salivary gland extracts do interfere with the normal coagulation process as demonstrated by the recalcification time assay. Using this as a baseline, the effects of saliva on recalcification time, activated partial thromboplastin time, prothrombin time, and thrombin time were measured to determine which arm(s) of the coagulation cascade might be impacted. Factor-deficient plasma assays also were used to measure possible perturbations in clotting. Gland-free saliva delayed the recalcification time as well as the activated partial thromboplastin time, prothrombin time, and thrombin time. Saliva also further delayed clotting times of plasmas deficient in factor V, factor VIII, and factor XIII, indicating that other factors in the coagulation cascade were inhibited. Although horn fly saliva did not alter the ability of deficient plasma reconstituted with factor X to clot, it did inhibit deficient plasma reconstituted with factor II (thrombin). Antithrombin activity in saliva was confirmed by its ability to interfere with thrombin hydrolysis of fibrinogen, its normal substrate, and by its inhibition of thrombin action on a chromagenic substrate that mimics the hydrolytic site of fibrinogen. Thus, horn fly saliva contains a factor that specifically targets thrombin, a key component in the coagulation cascade. While the biochemical mechanisms of inhibition may vary, this antihemostatic characteristic is shared with other zoophilic Diptera such as black flies, Simulium spp., and tsetse, Glossina morsitans morsitans Westwood, that feed on ungulates.

Animals↗
PubMed1990

Congenital coagulation protein deficiencies in the perinatal period.

Less common than acquired bleeding disorders in the perinatal period, congenital deficiencies of the clotting proteins, particularly the hemophilias A and B, can be just as devastating. Detection of these disorders may be delayed because a suspect family history is lacking in up to one third of the cases of hemophilia, and parents are usually asymptomatic in the other congenital factor deficiencies. Physiologically low levels of the liver-dependent and contact factors can make the interpretation of the common hemostasis screens difficult. Hence, the use of age-adjusted tables such as those presented here is essential. Congenital deficiencies of clotting proteins should be suspected when serious CNS bleeding occurs in an otherwise healthy baby generally after a day or two. Factor XIII or other less common factor deficiencies should be sought when there is prolonged bleeding from the umbilical stump. Bleeding after circumcision is a rare early indicator nowadays with better techniques. The availability of bedside ultrasound to detect CNS hemorrhage early and of fresh frozen plasma for treatment preceding the results of specific factor assays should prevent serious sequelae. Elective cesarean section is currently recommended when an unborn infant is known to have hemophilia.

Blood Coagulation Disorders↗
PubMed2005

Safety, pharmacokinetics, and immunogenicity of single-dose rFXIII administration to healthy volunteers.

BACKGROUND: Factor XIII (FXIII) is a transglutaminase that cross-links fibrin and other proteins to improve clot strength and resistance to fibrinolysis. Both congenital and acquired FXIII deficiency may result in a bleeding diathesis, and plasma-derived FXIII has been used to treat many of these clinical conditions. OBJECTIVES: A clinical study was designed and performed to evaluate the safety, pharmacokinetics, and immunogenicity of recombinant FXIII (rFXIII) administration to healthy adult volunteers. PATIENTS AND METHOD: Fifty healthy adult volunteers were enrolled in this randomized, double-blinded, placebo-controlled study. A single dose of rFXIII, ranging from 2 U kg(-1) to 50 U kg(-1), or placebo was administered. Safety was evaluated by capturing adverse events, clinical safety laboratory studies, and clinical score for deep venous thrombosis. Blood samples were taken for pharmacokinetic and immunogenicity analysis throughout the 28-day follow-up period. RESULTS: Recombinant FXIII was well tolerated, with no serious adverse events or dose-related toxicities. Following a single i.v. injection of 50 U kg(-1) rFXIII, the estimated terminal half-life was 270-320 h, the volume of distribution ranged from 40 to 75 mL kg(-1), and FXIII activity increased 1.77% per 1 U kg(-1) rFXIII administered. Increase in circulating A2B2 and decrease in free FXIII-B subunit indicate in vivo formation of FXIII heterotetramer. An immunogenic response to rFXIII or yeast, the production host, was not observed. CONCLUSIONS: Recombinant FXIII was well tolerated at doses of up to 50 U kg(-1) in healthy adult volunteers. The safety, pharmacological and immunological profile of rFXIII suggests it should be studied in patients with congenital FXIII deficiency as well as evaluated as a systemic hemostat in patients with acquired FXIII deficiency or hemorrhage.

Adolescent↗
PubMed1998

Factor XIII insufficiency in a patient with severe psoriasis vulgaris, arthritis, and infirmity.

Factor XIII (FXIII) links soluble fibrin monomers and collagen fibres to stable fibrin connections. Deficiency of FXIII, caused by dyspoiesis or increased consumption, results in a bleeding tendency and wound healing complications. Although the decrease of FXIII and successful replacement in patients with wound healing complications after surgery have been described by several authors, it is rarely considered that patients with autoimmune diseases, bleeding or healing complications may suffer from FXIII deficiency. We report a patient with severe psoriasis vulgaris generalisata with large, painful erythemas, bleeding tendency, joint contractions and infirmity, whose FXIII activity was 19%. After successful replacement the bleeding tendency vanished, and a marked improvement of skin and joint mobility allowed mobilisation and administration of physical therapy, whereby some independence and mobility were restored to the patient.

Adult↗