Sepsis is associated with the activation of a number of host defence mechanisms that lead to profound metabolic changes. The haematological changes primarily involve leucocytes, platelets and the haemostasis system. The well-known leucocyte alterations entail neutrophilia or neutropenia which are directly related to the ongoing infection. These will not be discussed in this article. This review...

Factor IX is a zymogen enzyme of the blood coagulation cascade. Inherited absence or deficit of the IX functional factor causes bleeding disorder hemophilia B, which requires constant protein replacement therapy. Reviewed herein are the current state in the manufacturing of FIX, improved variants of the recombinant protein for therapy, transgenic organisms for obtaining FIX, and the advances in...

Myocarditis is a potentially life-threatening disease. Although ventricular thrombus formation in myocarditis is rare, it carries the risk of serious complications. We report on a 10-year-old previously healthy girl presenting with 2 large left ventricular thrombi in acute lymphocytic acute myocarditis. No coagulation disorder was found. Her clinical course and mobile thrombi characteristics pr...

Factor V deficiency is a relatively uncommon disorder, inherited as an autosomal recessive trait that manifests clinically only in individuals who inherit the defective gene from both parents. The hemorrhage of nasal and oral cavity and ecchymosis are common but intracranial hemorrhage is very rare. We experienced a 53 year old male patient with intracranial hemorrhage due to factor V deficienc...

We describe a case of acute lumbar epidural hematoma at the L2-3 level complicated by paraplegia, which occurred after coagulation disorder because of massive bleeding intraoperatively in cesarean section. The preoperative coagulation laboratory finding was in normal range and so we tried combined spinal epidural anesthesia. Uterine atony occurred in the operation, and there was persistant blee...

BACKGROUND Disseminated Intravascular Coagulation (DIC) is an acquired syndrome characterized by systemic intravascular activation of coagulation, leading to deposition of fibrin in the circulation, occurring in the course of severe diseases. OBJECTIVE To review literature for articles that focus on the pathogenesis, diagnosis, and management of DIC. INFORMATION SOURCES Selected articles fr...

An 11-month-old girl presented to hospital with a massive subdural haematoma and bilateral retinal haemorrhages following an allegedly minor fall. There were no external signs of bruising and no prior bleeding tendency was reported. Although initial analyses were normal, repeated testing of the coagulation-fibrinolysis system led to a diagnosis of mild von Willebrand disease (vWD) Type 1. It wa...

PURPOSE To describe the first case of anterior segment dysgenesis associated with factor VII congenital deficit (hypoproconvertinemia). METHOD A 2-month-old child with factor VII deficiency was referred to our clinic because of corneal opacities. The child was born to consanguineous parents and severe factor VII deficiency was diagnosed on the third day of life because of gastrointestinal ble...

Budd Chiari syndrome or hepatic venous outflow obstruction is a complex entity with multiple etiologies and various clinical manifestations. It is often difficult to establish the diagnosis. The most common cause is a hypercoagulable state due to either genetic disorders of blood coagulation or several acquired conditions such as hematological diseases, tumors, infections, chronic inflammatory ...

A III (AT-III) is a liver-synthesized plasma glycoprotein of moderate size (58 kD) that can inhibit most of the activated serine proteases involved in coagulation (eg. thrombin. activated factor X. VII. etc). The inactivation involves formation of an irreversible complex. probably filtered from the circulation of the RES. The complex formation is a time-dependent reaction requiring minutes for ...