UW 5 Flashcards
Hemophilias (clasification)
Deficiency:
A=VIII (most common) XR
B= IX. XR
C= X. AR
Hemophilia diagnosis
PPT is prolonged. PT and BT normal
Best initial test: Patients blood + normal plasma = correction on PTT
Most accurate: Obtain specific factor deficiency. Look for 8, 9, 10, 11 and 12
Treatment of hemophilias
Depends on severity of defect
if <1% of normal: inmidiate factor transfusion
if >5% (mild) or 1%-5% (moderate): desmopresin===> factor 8 release
Prophylactic application of clotting factor concentrates is the basis of modern treatment for severe hemophilia A.
Functions of wWF (3)
- Bring platelets to exposed endothelium
- Transport/bind factor VIII
- Aggregate platelets
Labs of vWF deficiency and diagnosis
Increased bleeding time. PTT (normal or elevated) normal PT
Ristocetin cofactor essay (messures vWF capacity to aggregate platelets)
Treatment of vWF deficiency
Mild
Severe
Prevention
Desmopressin for mild cases
Factor VIII concentrate or vWF for severe cases
Control bleeding with OCPs, avoid ASAs and NSAIDs and platelet function inhibitor
Pathophysiology Factor V Leiden
Factor V is resistant to cleavage by protein C
Increased risk for DVT and PE is 5 fold if heterozygous and 50fold if homozygous
Young, white patient with personal or family hx of DVT/PE in unusual locations
Heparin-induced thrombocytopenia
Platalet agregation and a >30% decrease of platelet count after the administration of heparin (most common with unfractionated)
5-10 after. Can present with necrosis at injection site
Venous (most common) and arterial thromboembolism can occur
Diagnostic tests for:
Factor V leiden
Heparin-induced thrombocytopenia
Antiphospholipid sd
a. APC resistance test
b. Platelet factor 4 antibody or serotonin release assay
c. lupus anticoaguland and anticardiolipin
Managment of thrombophilias
Heparin-Warfarin for 3-6 months on 1st time event
Lifelong anticoagulation for >2 events
Vena cava filter if anticoagulation is contraidicated
Management for Heparin-induced thrombocytopenia
Stop heparin
Direct thrombin inhibitors (fondaparinux, argatroban and bivaluridin)
Switch to Warfarin
Labs in DIC
Cause by Fibrin deposition in small blood vessels. Present with both thrombosis and hemorrhages
Elevated PT and PTT
Low platelets
Elevated D-dimer
Eleveted fibrin, low fibrinogen
Presentation of TTP and HUS
LMNOP
Low platelet count
Microangipathic hemolytic anemia (jaundice, pruritus)
Neurological sings (seizures, stroke, confusion, decrease vision)
Obsolete renal function (AKI)
Pirexia
HUS does not present with neurologic sings and is more common in children with E. coli infection O157:H7
Common associations with Thrombotic trombocytopenic purpura (8)
SLE Malignancy Pregnancy Cyclosporine, quinidine, clopidogrel, ticlopidine AIDS
Labs in TTP and HUS
Low platelets, low hemoglobin, increased indirect bilirrubin
Normal clotting/bleeding labs
Schystocytes on smear
