Antiphospholipid (aPL) syndrome (APS) is an autoimmune thrombophilic syndrome for which definitive disease mechanisms have not been established. The condition is defined and diagnosed by a constellation of clinical and laboratory criteria, detailed below. This chapter reviews the current understanding…
Arterial and venous thromboses are common problems for all clinicians. Some patients with thrombosis have an underlying hypercoagulable state. These hypercoagulable states can be divided into three categories: inherited disorders, acquired disorders, and those that are mixed in origin. Inherited…
A variety of disorders, including infectious or inflammatory conditions and malignant disease, will lead to activation of coagulation. In many cases, this activation of coagulation will not lead to clinical complications and will not even be detected by routine laboratory…
This chapter reviews products available to treat deficiencies of plasma coagulation proteins. The development of blood component therapy and subsequently protein concentrates that are enriched in particular coagulation factors and other proteins made possible the effective treatment of bleeding episodes…
The term “rare coagulation factor deficiency” refers to disorders of thrombin and/or fibrin formation caused by mutations in a single gene, other than those for von Willebrand factor (see Chapter 133 ), factor VIII, or factor IX (see Chapter 134…
Hemophilia is the most common, severe inherited bleeding disorder recognized in humans. The X-linked mode of inheritance has been appreciated since biblical times, and the previous occurrence of the disease in the European royal family has added further interest in…
Von Willebrand factor (VWF) is an adhesive multimeric plasma glycoprotein (GP) that mediates platelet adhesion to injured subendothelium via GPIbα, and binds and stabilizes factor VIII (FVIII) in the circulation, protecting it from proteolytic degradation. This important multifunctional protein was…
In 1924, Moschowitz reported a case of a 16-year-old girl who died of a previously undescribed illness characterized by microangiopathic hemolytic anemia (MAHA), petechiae, hemiparesis, and fever. Postmortem examination revealed numerous hyaline thrombi, most prevalent in the terminal arterioles and…
Heparin-induced thrombocytopenia (HIT) is the most important drug-induced immune-mediated cytopenia for several reasons. First, heparin is a widely used anticoagulant (see Chapter 143 ). Second, HIT is relatively common, occurring in approximately 1% to 3% of postoperative patients, and 0.2%…
Thrombocytopenia is defined as a platelet count below the lower limit of the normal range (≈150 × 10 9 /L). Sometimes, an expanded definition of thrombocytopenia is appropriate. For example, an abrupt drop in the platelet count can signify the…