Conditions Associated with Decreased Coagulation

Hemophelia

• Manifestations

  • may present as a mild, moderate or severe bleeding disorder

  • excessive bleeding with trauma or surgery

  • bleeding into soft tissues, muscles and joints

  • excessive menstrual bleeding

• Treatment

  • avoidance of injury, prevention of bleeding

  • replacement therapy with recombinant clotting factors

Von Willebrand Disease

• Manifestations

  • Most common hereditary bleeding disorder

  • A genetic lack of vWF | a reduction in platelet adhesion

• Symptoms

  • Excessive bruising

  • Bleeding | mild to moderate

  • Occurence of bleeding | gums, nose, GI tract

  • Heavy blood flow | menstruation

Desmopressin Acetate

• one of the treatments for von Willebrand disease

• a vasopressin analog

• Increases activity of factor VIII, stimulates production of vWF

Vitamin K

• an essential factor for synthesis of factors II, VII, IX, and X by liver enzymes

• Source: green leafy vegetables | through bacterial metabolism in the gut

Liver Disease

• Site of synthesis of clotting factors: Liver

• Any disease or condition that alters liver function may lead to defective production of clotting factors

Thrombocytopenia

• any condition in which platelet count is abnormally low (<100,000 microL/blood)

Immune Thrombocytopenia Purpura

• Occurence

  • children and adolescents following a viral infection

  • autoimmune disorder in which antibodies bind to platelets in circulation

  • Enlargement of spleen

Antiphospholipid Syndrome

• Hypercoagulability caused by the formation of antibodies against negatively charged phospholipids

• the abnormal antibodies that are produced appear to activate the coagulation cascades

Hypercoagulability

• individuals with _______ are at increased risk for venous thrombus and emboli

Leiden Mutation

• genetic defect in clotting factor V that makes it less susceptible to inactivation

• affects 2%-5% of whites

Disseminated Intravascular Coagulation

• A secondary condition that can occur as a result of overactivation of the intrinsic and extrinsic coagulation pathways

• Characterization: Paradoxical of both inappropriate activation of the clotting cascades and the distruction of platelets

Anemia

• a condition in which there is a reduced number of red blood cells or decreased concentration of hemoglobin in those cells or both

Hemolytic anemia

• anemia that results from excess destruction of red blood cells (hemolysis)

Iron-deficiency anemia

• major cause of anemia worldwide

• Causes:

  • iron-deficient diets

  • poor absorption of iron from the intestine

  • persistent blood loss | ulcers, neoplasia

Cobalamin-deficiency or Folate deficiency anemia

• Vitamin B12 and folic acid are essential nutrients required for DNA synthesis and red bloock cell maturation

• Deficiency results to:

  • formation of abnormal shaped-red blood cells with shortened life spans due to weakened cell membranes

Sickle cell disease

• is a group of autosomal recessive disorders characterized by abnormal hemoglobin production

Thalassemia

• a genetic disorder characterized by absent or defective production of hemoglobin a or B chains

B thalassemia

• defective formation of B hemoglobin chains

• most common in individuals from Mediterranean populations

a Thalassemia

• defective formation of alpha-hemoglobin chains

• occurs mostly in asians

G6PD

• an enzyme that helps protect red blood cells against hemolysis during periods of oxidative stress

• found on the X-chromosome

G6PD Deficiency

• a defective gene for G6PD | fully expressed in homozygous males | partially expressed in heterozygous females

• most common in patients of Mediterranean or African-American descent

• individuals are usally asymptomatic

Aplastic Anemia

• caused by a lack of red blood cell production by the bone marrow

• if erythrocyte stem cells precursors are lacking or destroyed, the process of erythropoeisis will be severely impaired

Polycythemia

• is a disorder in which the number of red blood cells in circulation is greatly increased

Relative polycythemia

• results from an increase in the concentration of red blood cells due to a loss of plasma volume

Primary polycythemia

• polycythemia vera

• caused by excessive proliferation of bone marrow stem cells

Secondary polycythemia

• may occur from excess erythropoetin production as a physiologic response to hypoxia