Hemorrhagic diseases

Lesions of the blood vessels Lesions of the blood vessels Abnormal platelets Abnormal platelets Abnormalities in the coagulation cascade Abnormalities in the coagulation cascade Combinations of abnormalities Combinations of abnormalities

Lesions of the blood vessels Senile purpura Senile purpura *hemorrhage in the back of hands and forearms or older persons *atrophy of the vessels

SCURVY Vitamin C deficiency gingival bleeding( ANY mucous membrane) bleeding into muscles bleeding into subcutaneous tissues bleeding around hair follicles; corkscrew-like hair Normal collagen synthesis depends upon the hydroxylation of proline and lysine enzymes that catalyze the hydroxylation require ascorbic acid

Hemorrhage in the hair follicles; corkscrew hair in scurvy

Thrombocytopenia (low platelets count) Petechiae in the skin Oozing from mucosal membranes Bleeding within the brain Low platelet count Prolonged bleeding time

Causes of thrombocytopenia Drugs, chemicals Irradiation Leukemia Myelophthisis (tumor cells replace normal bone marrow cells) Splenic sequestration Multiple blood transfusions DIC

Idiopathic thrombocytopenic purpura ITP Antibodies against platelets Damaged platelets are removes by macrophages in the spleen Low platelets Normal/increased megakaryocytes

Thrombotic thrombocytopenic purpura TTP Hyaline aggregates in small blood vessels Low platelets Anemia (abnormal blood vessels trap the RBC) Renal, CNS abnormalities, fever Cause: von Willebrand disease, enzyme deficiency

Von WIllebrand disease: abnormal platelet adhesion Aspirin intake: low TxA2; abnormal platelet aggregation

Abnormalities in the Coagulation cascade Bleeding from larger vessels hemarthroses (joints) large hematomas/ ecchymoses extensive bleeding with trauma

Abnormalities in the Coagulation cascade Classic hemophilia Christmas disease Vitamin K deficiency

Classic hemophilia Hemophilia A Factor VIII deficiency X-linked Occurs worldwide Bleeding into the : muscles Subcutaneous tissues Joints “royal disease”

Christmas disease Factor IX deficiency Less common than Hemophilia A Same presentation

Vitamin K deficiency Adults: fat malabsorption in diseases of the pancreas or small intestines Infants: Hemorrhagic disease of the newborn - intestines have small amounts of bacteria -decrease factors II, VII, IX, X

Combination of abnormalities Von Willebrand disease DIC Coagulopathy of liver disease

Von Willebrand disease MOST COMMON HEREDITARY BLEEDING DISORDER Autosomal dominant Decreased in vWF: Decrease platelet adhesion to the injured blood vessel (prolonged bleeding time)

DIC Consumption of platelets and coagulation factors II, V, VIII Low fibrinogen Hemorrhage and thrombosis Anemia Cause: release of thromboplastin, triggers the intrinsic pathway of coagulation; increased fibrinolysis

DIC Obstetric complications Toxemia Amniotic fluid embolism retained dead fetus placental accidents Cancer – lung, pancreas, prostate, stomach Infection – bacteria Trauma Immune diseases

Coagulopathy of liver disease All coagulation factors are produced in the liver EXCEPT vWF Treatment: Vitamin K