Sickle cell disease (SCD) results from a single base pair change in the β-globin subunit, yet the downstream effects and complex manifestations that result are protean and variable. The abnormal HbS is insoluble when deoxygenated and polymerizes causing deformation of…
Disseminated intravascular coagulopathy (DIC) is defined as: An acquired syndrome characterized by the intravascular activation of coagulation with loss of localization arising from different causes. It can originate from and cause damage to the microvasculature, which if sufficiently severe, can…
Etiology and Pathogenesis Vascular Etiology The tumor microenvironment of many malignancies is rich with angiogenesis-stimulating growth factors; the most well-known is vascular endothelial growth factor (VEGF). The neoangiogenesis are typically unorganized and structurally dissimilar to the nonneoplastic vasculature and are…
Introduction Renal disease has long been associated with a bleeding diathesis; Morgagni described the association of uremia and bleeding as early as 1764. Although dialysis and erythropoietin-stimulating agents (ESAs) have greatly decreased this bleeding tendency, bleeding is still reported in…
Most of the hematologic complications that occur in cardiac disease are linked to the disturbances in the dynamics of flow. Although there are fewer hemorrhagic complications due to intrinsic cardiac disease than occur with cardiac surgeries and implantation devices, those…
Introduction In 1894, the condition known as hemorrhagic disease of the newborn was first discovered to be related to a deficiency of vitamin K (VK), which is an essential cofactor for the synthesis of clotting factors, FII, FVII, FIX, FX,…
Liver disease is associated with multiple hemostatic defects leading to both hemorrhagic and thrombotic manifestations. Its effect on coagulation factors, endogenous anticoagulants, and platelets results in rebalanced hemostasis and creates challenging clinical scenarios requiring careful management. Decreased Synthesis of Pro-…
Hereditary Vascular Malformations Hereditary Hemorrhagic Telangiectasia (Osler–Weber–Rendu Disease) Hereditary hemorrhagic telangiectasia (HHT) is a systemic autosomal dominant (AD) vascular disease that is characterized by mucocutaneous telangiectasias and multiple arteriovenous malformations (AVMs). It is the most common inherited vascular bleeding disorder…
Postpartum hemorrhage (PPH) is a leading cause of maternal death worldwide. Although the majority of PPH occurs as a result of uterine atony or other anatomical defect, the risk is increased when a congenital or acquired hemostatic defect is present.…
Factor XIII (FXIII), α 2 -antiplasmin (α 2 -AP), and plasminogen activator inhibitor type 1 (PAI-1) deficiencies are all very rare bleeding disorders. These proteins play a critical role in stabilizing a fibrin clot (FXIII) and regulating fibrinolysis through the…