[Haemostatic abnormalities and thyroid disorders: the prospective observational MITH study].

Haemostatic abnormalities are a common phenomenon in patients with thyroid diseases. On one hand the condition of hyperthyroidism is associated with an increased risk of thrombotic events, on the other in severe hypothyroidism can be found a haemorrhagic tendency, as opposed to the subclinical hypothyroidism seems to correlate with increased thrombotic risk. The prospective, single center, observational MITH study (Mantua Investigation on Thyroid and Haemostasis), whose results are presented, aims to evaluate coagulation parameters in patients with thyroid disease, to establish the prevalence of haemostatic abnormalities in various conditions, to analyse the implications and clinical response to therapy established.

[Clinical use of virally inactived plasma. The experience of Blood Transfusion Unit in Mantova, Italy].

Background: Fresh Frozen Plasma (FFP) is a blood component whose clinical use is widespread worldwide. Transfusion safety of this product is ensured by legally obligatory tests. Although these tests are carried out on each plasma donation, safety levels can be further improved by using some technical procedures, such as, among others, methylene blue (MB) and solvent-detergent (SD) viral inactivation methods.

The spectrum of coagulation abnormalities in thyroid disorders.

The hemostatic balance is a complex system where the delicate equilibrium is regulated by several factors including hormones. A variety of endocrine disorders have been reported to be associated with coagulation abnormalities, ranging from mild laboratory changes to clinically relevant thrombotic or bleeding manifestations. In this review, we summarize the current knowledge on the main abnormalities of the coagulation and fibrinolytic systems associated with thyroid dysfunctions.

Inherited and acquired factor V deficiency.

The clotting factor V, also known as proaccelerin or labile factor, is synthesized by the liver and possibly by the megakaryocytes. Factor V exerts a pivotal role in hemostasis, as it participates in both procoagulant and anticoagulant pathways, being an essential cofactor of the prothrombinase complex in the former case and participating in the inactivation of factor VIII (FVIII) in the latter. Isolated factor V deficiency due to mutations in the F5 gene is a rare inherited coagulopathy typically associated with a broad spectrum of bleeding symptoms, ranging from easy bruising, delayed bleeding after haemostatic challenges such as trauma or surgery to more severe joint bleeds.

Protein C concentrate as adjuvant treatment in neonates with sepsis-induced coagulopathy: a pilot study.

The objective of the study is to describe safety and effects of protein C concentrate (PCConc) administration in neonates with sepsis-induced coagulopathy. Eighteen neonates (12 preterm and 6 full term) aged between 1 and 28 days who have severe sepsis (n = 6) or septic shock (n = 12), with coagulopathy and acquired protein C (PC) deficiency received PCConc (i.v.

Prophylaxis in congenital hemophilia with inhibitors: the role of recombinant activated factor VII.

The development of inhibitors against therapeutically administered factors VIII or IX is actually the most challenging complication of hemophilia patients with inhibitors. The introduction of bypassing agents (i.e.

Hemostatic abnormalities in endocrine and metabolic disorders.

The hemostatic balance is a complex system where the delicate equilibrium is regulated by several factors, including hormones. This review summarizes current knowledge of the effects of most frequent endocrine and metabolic diseases (such as hypothyroidism, hyperthyroidism, Cushing's syndrome, GH-related pituitary dysfunctions, pituitary prolactin-producing adenomas, polycystic ovary syndrome, primary hyperparathyroidism, and metabolic syndrome) on coagulation and fibrinolysis. Overt hypothyroidism appears to be associated with a bleeding tendency, whereas all other endocrine diseases appear to be associated with a thrombotic tendency.

The use of desmopressin in congenital factor XI deficiency: a systematic review.

Factor XI (FXI) deficiency is a rare inherited coagulation disorder characterized by infrequent spontaneous bleeding, but increased risk of hemorrhagic complications especially after trauma or surgery. Treatment options for FXI-deficient patients include virus-inactivated fresh frozen plasma, plasma-derived FXI concentrates, and activated recombinant FVII. Inhibitors of fibrinolysis, such as tranexamic acid, and desmopressin (DDAVP) have also been used in these patients, especially in mild cases.

Interpatient phenotypic inconsistency in severe congenital hemophilia: a systematic review of the role of inherited thrombophilia.

It is well known that the clinical phenotype of hemophilia may vary greatly among patients with the same apparent level of coagulation factor and the same genetic mutation. Thus, patients with severe hemophilia may experience a severe phenotype or only a milder bleeding tendency, suggesting some other moderating influence. To elucidate the mechanism of this heterogeneity, some investigators have recently suggested that inherited thrombophilic factors may play a role in the milder clinical presentation of severe hemophilia.

Thyroid dysfunction and hemostasis: an issue still unresolved.

Thyroid hormones exert various effects on the hemostatic system, as documented by the fact that subclinical or overt thyroid dysfunctions may be associated with hypocoagulable or hypercoagulable states. In this review, the hemostatic balance (primary hemostasis, coagulation factors, and fibrinolytic system) in different thyroid disorders is analyzed from a laboratory, pathogenic, and clinical point of view. Although limited, the published studies suggest that patients with hyperthyroidism or subclinical hypothyroidism have an increased thrombotic risk, whereas patients with overt hypothyroidism have a bleeding tendency.

Extracorporeal immunoadsorption for the treatment of coagulation inhibitors.

Extracorporeal immunoadsorption is a widely used technique for the removal of pathogenic antibodies in a variety of immunologic disorders. This procedure has been used in patients with high-titer inhibitors against coagulation factors for the temporary removal of antibodies before initiating replacement therapy to achieve hemostasis and stop acute bleeding or to cover a surgical procedure. Inhibitor removal by immunoadsorption has also been included at the onset of immune tolerance protocols in both acquired and congenital hemophilia.

Thyroid-associated autoimmune coagulation disorders.

Abnormalities of blood coagulation are not rarely observed in patients with thyroid dysfunctions and may range from subclinical laboratory abnormalities to clinically significant hemorrhagic or thrombotic complications. In this review, we summarize the current knowledge on thyroid-associated autoimmune coagulation disorders (i.e.

Performance of the automated and rapid HemosIL D-Dimer HS on the ACL TOP analyzer.

We evaluated the analytical performance of the new commercial HemosIL D-Dimer HS, a latex-enhanced turbidimetric immunoassay, from Instrumentation Laboratory for D-dimer measurement on the ACL TOP automated analyzer. The recommended cut-off for this immunoassay is 243 ng/ml. The within-run and between-run coefficients of variations of D-Dimer HS for low, intermediate and high D-dimer concentrations were: 3.

Acquired factor VIII inhibitors in oncohematology: a systematic review.

Acquired hemophilia A is an uncommon but potentially life-threatening hemorrhagic disorder caused by the onset of autoantibodies against coagulation factor VIII. Acquired hemophilia A is most frequently associated with autoimmune diseases, neoplasia, pregnancy and drug reactions but in approximately 50% of the cases no underlying disorder can be identified. A prompt diagnosis of this acquired bleeding disorder is essential for the appropriate management which is aimed to the control of hemorrhage and the suppression of inhibitor.

Potential role of recombinant activated factor VII for the treatment of severe bleeding associated with disseminated intravascular coagulation: a systematic review.

Recombinant activated factor VII (rFVIIa) is a novel hemostatic agent, originally developed for the treatment of hemorrhage in hemophiliacs with inhibitors, which has been successfully used recently in an increasing number of nonhemophilic bleeding conditions. In the present systematic review we report the existing literature data on the use of this hemostatic agent in severe bleeding, unresponsive to standard treatment, associated with disseminated intravascular coagulation. A total of 99 disseminated intravascular coagulation-associated bleeding episodes treated with rFVIIa were collected from 27 published articles: in the majority of the cases, the underlying disorder complicated by disseminated intravascular coagulation was a postpartum hemorrhage, while in the remaining cases it was a cancer, trauma, sepsis or liver failure.

Influence of the centrifuge time of primary plasma tubes on routine coagulation testing.

Preparation of blood specimens is a major bottleneck in the laboratory throughput. Reliable strategies for reducing the time required for specimen processing without affecting quality should be acknowledged, especially for laboratories performing stat analyses. The present investigation was planned to establish a minimal suitable centrifuge time for primary samples collected for routine coagulation testing.

Pathogenesis, clinical and laboratory aspects of thrombosis in cancer.

The relationship between increased clotting and malignancy is well recognized, though the bidirectional development of this association is often overlooked. In the challenging cancer biology, transforming genes often act in concert with numerous epigenetic factors, including hypoxia, inflammation, contact between blood and cancer cells, and emission of procoagulant vesicles from tumors, to determine a net imbalance of the hemostatic potential which is detectable by a variety of laboratory tests. Procoagulant factors, in particular, are intimately involved in all aspects of hemostatic, cell proliferation and cellular signalling systems.

Drug-induced anti-factor VIII antibodies: a systematic review.

Acquired hemophilia A is an uncommon but potentially life-threatening hemorrhagic disorder caused by the onset of autoantibodies against coagulation factor VIII. Acquired hemophilia A is most frequently associated with autoimmune diseases, solid tumors, lymphoproliferative diseases, pregnancy, and drug reactions. However, in approximately 50 percent of the patients no underlying disorder can be identified.

Increased factor VIII coagulant activity levels in male carriers of the factor V R2 polymorphism.

A common factor V gene haplotype, the FVR2 haplotype (FVHR2), has been associated with a reduced cofactor activity in activated protein C-mediated activated factor VIII inactivation. Our aim was to investigate the role of FVHR2 as a possible determinant of factor VIII levels in a population study. A total of 516 individuals (401 men, 115 women; mean age 58.

High prevalence of inherited prothrombotic risk factors in 134 consecutive patients with von Willebrand disease.

We screened 134 consecutive patients with von Willebrand disease (VWD) (106 type 1, 26 type 2, and 2 type 3 VWD) for the most important inherited prothrombotic risk factors. One hundred eight patients (80.6%) were positive for at least one of the prothrombotic risk factors screened for.

Inherited thrombophilia.

Inherited thrombophilia can be defined as a genetically determined predisposition to the development of thromboembolic complications. Since the discovery of activated protein C resistance in 1993, several additional disorders have been described and, at present, it is possible to identify an inherited predisposition in about 60 to 70% of patients with such complications. These inherited prothrombotic risk factors include qualitative or quantitative defects of coagulation factor inhibitors, increased levels or function of coagulation factors, defects of the fibrinolytic system, altered platelet function, and hyperhomocysteinemia.

Recent acquisitions in the pathophysiology, diagnosis and treatment of disseminated intravascular coagulation.

Disseminated intravascular coagulation (DIC) is a disorder characterized by both acute generalized, widespread activation of coagulation, which results in thrombotic complications due to the intravascular formation of fibrin, and diffuse hemorrhages, due to the consumption of platelets and coagulation factors. Systemic activation of coagulation may occur in a variety of disorders, including sepsis, severe infections, malignancies, obstetric or vascular disorders, and severe toxic or immunological reactions. In this review, we briefly report the present knowledge about the pathophysiology and diagnosis of DIC.