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Hematologic Changes in Pregnancy

Hematologic conditions are often seen during pregnancy as part of normal physiology and complications of varying severity. The primary physiologic, hematologic changes during pregnancy relate to the expansion of plasma volume and the prothrombotic state that develops as the pregnancy advances. They are thought to prepare the mother and fetus for eventual placental separation. Understanding the complex physiology of pregnancy and the interplay with hematology facilitates…

Bleeding And Clotting Disorders In Pediatrics

The neonatal and pediatric stages are periods of rapid physiologic changes, some of which affect the hemostatic system. The hemostatic system is a dynamic system that evolves gradually from birth into the mature adult form. Evaluation of bleeding and clotting disorders in children requires an understanding of the evolution of physiologic normal values for age, the congenital disorders that present in early life, and the common…

Atrial Fibrillation

Atrial fibrillation (AF) is the most prevalent sustained cardiac arrhythmia, affecting approximately 3% of adults worldwide. Its prevalence and incidence are rising continuously with the increasingly aging population. AF is associated with impaired prognosis, increased risk of all-cause mortality, stroke, heart failure, and hospital admissions, in addition to poor quality of life. Importantly, AF should not be considered in isolation regarding the process of prevention, detection,…

Peripheral Artery Disease

Peripheral artery disease (PAD) is an important manifestation of systemic atherosclerosis with significant morbidity and mortality.PAD affects the lower extremities and is defined as a stenosis or occlusion in the aorta or the arteries supplying blood to the legs, including the iliac, femoral, popliteal, or infrapopliteal vessels (peroneal, posterior tibial, and anterior tibial arteries). Stenosis is typically caused by atherosclerosis. Nonatherosclerotic causes of vascular disease also…

Acute Coronary Syndromes

Acute coronary syndrome (ACS) leads to millions of hospital admissions worldwide each year and is a leading cause of death. Antithrombotic therapies are a cornerstone in the immediate and long-term management of ACS, reducing the risk of myocardial infarction (MI) and death in both medically and invasively managed patients. This chapter reviews fibrinolytic, antiplatelet, and anticoagulant therapies in the treatment of patients with ACS and provides…

Stroke

Stroke is the second leading cause of mortality worldwide and a leading cause of acquired adult disability. Primary stroke subtypes include ischemic stroke, intracerebral hemorrhage (ICH), and subarachnoid hemorrhage (SAH). Each stroke subtype has differing etiologies, outcomes, and management strategies. In the past 30 years, there have been major advances in the diagnosis (emergence of widely available neuroimaging) and treatment of acute stroke. In addition, there…

Antithrombotic Drugs

Arterial or venous thromboembolism is a major cause of morbidity and mortality. Arterial thrombosis is the most common cause of acute myocardial infarction, ischemic stroke, and limb gangrene, whereas deep vein thrombosis can lead to pulmonary embolism, which can be fatal, and to post-thrombotic syndrome. Most arterial thrombi are superimposed on disrupted atherosclerotic plaque because plaque rupture exposes thrombogenic material in the plaque core to the…

Atherothrombosis

Morbidity and mortality from atherosclerosis, the pathologic process underlying acute myocardial infarction, sudden death, stroke, and limb loss, represent an enormous burden on society and health care systems (see Chapter 144, Chapter 145, Chapter 146 ). Even though death rates from heart attack and stroke have dropped precipitously over the past 48 years (68% and 77%, respectively), cardiovascular disease (CVD) is still the number one cause…

Prevention and Treatment of Venous Thromboembolism in Pregnancy

Pregnant persons and those who are postpartum are at increased risk of venous thromboembolism (VTE). Although pregnancy-related VTE can affect any part of the venous circulation, as in the non-pregnant population, it most commonly manifests as deep vein thrombosis (DVT) or pulmonary embolism (PE). Management of pregnancy-related VTE is complicated as the diagnosis, prevention, and treatment of pregnancy-related VTE must consider fetal, as well as maternal,…

Venous Thromboembolism

Venous thromboembolism (VTE) encompasses deep vein thrombosis (DVT) and pulmonary embolism (PE). DVT commonly affects the deep veins of the legs, and infrequently involves veins at other anatomical sites (such as upper extremity, splanchnic, or cerebral veins). Thrombotic occlusion of the deep veins impairs drainage of blood, thereby resulting in pain and swelling distal to the obstruction. Embolization of thrombus from deep veins in the leg…

Antiphospholipid Syndrome

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 of pathogenic mechanisms, the current diagnostic tests for the condition, its clinical manifestations, and treatment approaches. Definition of Antiphospholipid Syndrome APS is an autoimmune thrombophilic condition…

Hypercoagulable States

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 hypercoagulable states, also known as thrombophilic disorders, can be due to loss of function of natural anticoagulant pathways or gain of function in procoagulant pathways (…

Disseminated Intravascular Coagulation

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 tests, but can only be measured with sensitive molecular markers for activation of coagulation factors and pathways. However, if activation of coagulation is sufficiently strong, the…

Transfusion Therapy for Coagulation Factor Deficiencies

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 in patients with hemophilia and other diatheses. In the 1940s, a collaborative effort funded by the US government was undertaken among protein scientists with the goal…

Rare Coagulation Factor Deficiencies

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 ), that reduce plasma activity of one or more coagulation proteins. The most common inherited deficiencies that cause defects in plasma coagulation are those for factor…

Hemophilia A and B

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 this condition. Hemophilia is subdivided into hemophilia A and B; the former being a deficiency or absence of factor (F) VIII while the latter is a…

Structure, Biology, and Genetics of von Willebrand Factor

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 named after the Finnish physician Dr. Erik von Willebrand, who first described von Willebrand disease (VWD) in 1926. In the original publication, he described a severe…

Thrombotic Thrombocytopenic Purpura and the Hemolytic Uremic Syndromes

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 capillaries of the heart and kidneys. In 1936, four similar cases were reported by Baehr and colleagues, who proposed that the hyaline thrombi were secondary to…

Heparin-Induced Thrombocytopenia

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% to 0.5% of medical patients, who receive unfractionated heparin (UFH) derived from porcine intestine for 7 to 14 days. Third, HIT frequently causes life- and limb-threatening…

Thrombocytopenia Caused by Hypersplenism, Platelet Destruction, or Surgery/Hemodilution

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 onset of a disorder such as heparin-induced thrombocytopenia (HIT) or bacteremia even if the platelet count remains above 150 × 10 9 This is especially…