Name the disorder characterized by: 1. Autoimmune IgG against platelet antigens is GP2B-3A 2. Thrombocytopenia due to ab-bound platelets being consumed by splenic macrophages 3. Acute and Chronic versions --> acute - children --> chronic - usually women of childbearing age; can be associated with SLE 4. Labs: Low platelets Normal PT/PTT High megakaryocytes in bone marrow 5. Treat with corticosteroids and splenectomy for refractory cases. IVIG used for short-term therapy.
ITP Immune Thrombocytopenic Purpura
Name the disorder characterized by: 1. Pathologic formation of platelet microthrombi in small vessels 2. Schistocytes 3. Associated with thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) 4. Labs: Thrombocytopenia Increased bleeding time Normal PT/PTT Anemia w/ schistocytes High megakaryocytes in bone marrow
Microangiopathic Hemolytic Anemia
Thrombotic Thrombocytopenic Purpura (TTP) is due to decreased _____, an enzyme that normally cleaves vWF into monomers for degradation.
ADAMS-TS-13 *CNS abnormalities common in TTP
Hemolytic Uremic Syndrome is classically seen in children with what infection?
E. Coli O157:H7 - dysentery from undercooked beef Verotoxin damages endothelial cells --> HUS --> Microangiopathic Hemolytic Anemia *Renal insufficiency common in HUS
Name the disorder characterized by: 1. Genetic GP1b deficiency 2. Impaired platelet adhesion 3. Blood smear: mild thrombocytopenia w/ enlarged platelets
Name the disorder characterized by: 1. Genetic G2b-3a deficiency 2. Impaired platelet aggregation
Glanzmann Thrombasthenia Glanzmann thrombasthenia is a rare autosomal recessive disorder with defective platelet aggregation from deficiency or dysfunction of glycoprotein IIb/IIIa.
T/F: Primary disorders of hemostasis do not involve the coagulation cascade, therefore PT/PTT will be normal.
Prothrombin time (PT) measures:
extrinsic factor (factor VII) and common pathways (factors II, V, X and fibrinogen) of the coagulation cascade
Partial Thromboplastin Time (PTT) measures:
intrinsic factor (factors XII, XI, IX, VIII) and common pathways (factors II, V, X and fibrinogen) of the coagulation cascade
Name the disorder characterized by: 1. Genetic Factor VIII deficiency (X-linked recessive, predominantly affects males) 2. Postsurgical, deep tissue and joint bleeding 3. Labs: Increased PTT normal PT Low factor VIII Normal platelet count Normal bleeding time 4. Treat with recombinant factor VIII
Hemophilia A *PTT does correct when mixed with normal plasma in Hemophilia A
Name the disorder characterized by: 1. Genetic factor IX deficiency 2. Postsurgical, deep tissue and joint bleeding 3. Labs: Increased PTT normal PT Low factor VIII Normal platelet count Normal bleeding time 4. Treat with recombinant factor XI
Name the disorder characterized by: 1. acquired antibody against a coagulation factor resulting in impaired factor, ie factor VIII 2. Clinical findings similar to Hemophilia A 3. PTT does not correct when mixed with normal plasma
Coagulation Factor Inhibitor
Name the disorder characterized by: 1. Genetic vWF deficiency 2. Labs: Increased bleeding time High PTT Normal PT Decreased factor VIII half-life Abnormal ristocetin test 3. Treatmet is desmopressin which releases vWF from Weibel-Palade bodies
Von Willebrand Disease
What is the most common inherited coagulation disorder?
Von Willebrand Disease
What is the ristocetin test?
Ristocetin induces platelet agglutination by causing vWF to bind to platelet gP1b. Lack of vWF (in Von Willebrand Disease) --> impaired agglutination --> abnormal test result
Name the disorder characterized by: 1. Multiple coagulation factor dysfunction 2. Deficiency in newborns 3. Can be induced by long-term antibiotic therapy 4. Can be found in malabsorptive processes 5. Can be found in alcoholics
Vitamin K deficiency
Name the disorder characterized by: 1. Platelet destruction secondary to heparin therapy 2. May cause thrombosis from destroyed platelets activating remaining platelets
Name the disorder characterized by: 1. Pathologic activation of the coagulation cascade 2. Widespread ischemia and infarcts 3. Usually secondary to another disease process ie obstetric problems, sepsis etc. 4. Can be caused by a rattlesnake bite 5. Labs: Thrombocytopenia Increased PT/PTT Low fibrinogen microangiopathic hemolytic anemia elevated D-dimer 6. Treat by addressing underlying cause and transfusing blood products
Disseminated Intravascular Coagulation (DIC)
Name the disorder characterized by: 1. Resembles DIC 2. Increased bleeding 3. Labs: increased PT/PTT increased bleeding time normal platelet count increased fibrinogen split products w/o d-dimer 4. Treatment is with aminocaproic acid which blocks activation of plasminogen
Disorders of fibrinolysis (removing the clot)
Causes of endothelial cell damage include:
1. Vasculitis 2. Atherosclerosis 3. High levels of homocysteine
Name the disorder characterized by: 1. Long, slender fingers 2. Mental retardation 3. Vessel thrombosis 4. Lens dislocation
Homocystinuria cystathionine beta synthase (CBS) deficiency resulting in high levels of homocysteine
Name the disorder characterized by: 1. DVTs at a young age 2. Excessive or defective procoagulant proteins
Proteins C and S normally inactivate factors:
5 and 8