Factor Problems Flashcards
Little spontaneous bleeding, but bleeding after surgery can be heavy, not as debilitating as hemophilia A
Afibrinogenemia
Afibrinogenemia Lab Results
PLT count normal
PT and PTT prolonged
Fibrinogen low
TT prolonged
Afibrinogenemia Treatment
Cryoprecipitate (contains factor 8 and fibrinogen)
alteration of the structure of fibrinogen, mild bleeding tendencies
Dysfibrinogenemia
Dysfibrinogenemia Lab Results
PLT Count normal
PT and PTT are prolonged
TT is prolonged
Fibrinogen normal
the patient is both over clotting and over fibrinolysing (most common)
Disseminated Intravascular Coagulation
Clinical Classification of DIC
Tissue Injury, Malignancy, Obstetrical, Infections
Clinical Manifestations of DIC
hemorrhaging from unrelated sites and thrombosis in intravascular of major organs
DIC Lab Reuslts
PLT low BT prolonged PT and PTT Prolonged Fibrinogen low TT prolonged D-dimer present
Therapy for DIC
Heparin, Cryo, Platelets
increased fibrinolysis, urokinase
primary fibrinolysis
Primary Fibrinolysis Lab Results
PT and PTT would be prolonged Fibrinogen low TT Prolonged FDP Increased D-dimer-
sex-linked spontaneous bleeding into joints and muscles
Hemophilia A
0-2% activity with spontaneous bleeding
Severe Hemophilia A
2-5% activity with bleeding from trivial injuries
Moderate hemophilia A
5-25% activity with bleeding from traumatic injuries
Mild Hemophilia A
Hemophilia Lab Reuslts
PTT prolonged
Factor Assay
.05 patient plasma and .05 normal plasma in the PTT to show that it is normal
Redo with a known depleted plasma until you see the test is prolonged
Shows the factor that is missing
Hemophilia Treatment
Factor VIII concentrates
Cryo - contraindicated
Recombinant factor VII
clinicial symptoms indistinguishable from hemophilia A
Hemophilia B - Christmas Disease
Hemophilia B Treatment
Proplex T
recombinant VII
easy bruising, bleeding from the gums and GI tract, heavy periods - mucosal bleeding
von Willebrand Disease
most common type of von Willebrand
VWF and VIII:C levels reduced - 70%
von Willebrands Lab Results
PTT prolonged
Ristocetin aggregation
Ristocetin cofactor assay
Ristocetin aggregation
PRP with ristocetin - abnormal test = von Willebrand’s
Ristocetin Co-Factor Assay
PPP with ristocetin and normal platelets to rule out Bernard Soulier’s
vWF Disease Treatment
Desmopressin (constricts the vessels)
Lupuslike Anticoagulants
prolonged PTT not corrected with mixing studies because of reaction against the phospholipids in the reagent
Hypercoagulation
associated with the inappropriate formation of thrombi in the vasculature
Factors of Hypercoagulation
Age, Immobilization, Elevated estrogens
Types of Hypercoagulation
Anti Thrombin Deficiency
Protein C Deficiency
Factor V Leiden mutation
Prothrombin variant
Protein C deficiency
normally inhibits factor V and VIII
Factor V Leiden
most recently described genetic defects, Protein C cannot inactivate; needs PCR
Prothrombin Variant
substitution of A for G at position 20210 to increase prothrombin
COX-2
made in the vascular endothelium, smooth muscle and some platelets
Plavix
blocks the P2Y12 ADP binding site on the platelet
