Bleeding Disorders Flashcards
Pathogenesis of Henoch-Sconlen purpura.
Vessel wall abnormalities - bleeding disorder
Widespread inflammatory reactions to vessels
Deposition of IgA, C3, and fibrin seen in skin biopsy
Clinical presentation of Henoch-Schonlein purpura.
Child <10
Following infection
Purpuric rash, GI bleed, glomerulonephritis, vasculitis, proteinuria
Normal platelet, bleeding time, and coagulation
Skin biopsy: IgA, C3 and fibrin
Causes/types of immune thrombocytopenic purpura.
Secondary: drug induced or leukemias Primary or idiopathic: acute or chronic -anti-platelet antibodies -thrombocytopenia, prolonged bleeding time -treat with corticosteroids
Clinical presentation of acute immune thrombocytopenic purpura.
Children
Follows viral infection often
Epistaxis and skin petechiae
Thrombocytopenia and prolonged bleeding time
Clinical presentation of chronic immune thrombocytopenic purpura.
Young to middle aged women
Bleeding from mucosal membranes
Easy bruising
Complications: intracranial and subarachnoid haemorrhages
Thrombotic thrombocytopenic purpura pathogenesis.
Deficiency of ADAMTS13
Excess vWF
Endothelial injury allows high platelet adhesion
Formation of thrombus
Thrombocytopenia and microangiopathic hemolytic anemia
Clinical presentation of thrombotic thrombocytopenic purpura.
Pentad: Fever Thrombocytopenia Renal failure Microangiopathic hemolytic anemia Neurological symptoms
Labs: Anemia, thrombocytopenia Prolonged bleeding time Immature RBCs Schistiocytes (sliced RBCs)
Lab of hemolytic uremic syndrome.
Proteinuria High serum creatinine and BUN EHEC O157:H7 Anemia and thrombocytopenia Schistocytes Prolonged bleeding time
The two bleeding disorders seen in consanguinity.
Bernard-Soulier syndrome
- defective platelet adhesions
- low platelets and giant platelets on smear
Glanzmann Thrombasthenia
- defective aggregation
- defective of fibrinogen receptor
- normal platelet count
Von Willebrand disease pathology.
Deficiency in vWF which is a platelet adhesion molecule
Carries factor VIII in plasma to site of injury
Ristocetin cofactors assay
Autosomal dominant
Prolonged bleeding time
Normal PT and platelets
Hemophilia A.
X linked Reduced factor VIII Normal platelets, bleeding time, PT Prolonged PTT Easy bruising, hemorrhage, no petechiae Hemorrhage to knee joints (hemarthroses)
Lab diagnosis of DIC.
Increased D-dimers Increased fibrinogen degradation products Decreased fibrinogen Prolonged PT, PTT, and TT Decreased platelets Schistocytes Toxic granulations in neutrophils
