Other Coagulation Disorders Flashcards
christmas factor
factor IX
the production of this is impaired by warfarin therapy
factors with vitamin k dependent activity
Hemophilia B
- Christmas disease
- X-linked recessive
- males usually affected; females are carriers
- anatomic, soft tissue bleeding
- mild to severe depending on sevrity of deficiency
treatment for hemophilia B
- factor IX concentrates
> recombinant factor IX
> prothrombin complex concentrates = vit K dependent coag factors - small percentage = develop an inhibitor/antibody
factor XI
- produced in liver
- circulates plasma complexed with HMWK
- contact factor in intrinsic pathway
> activated by XIIa and HMWK - only contact factor associate with bleeding problems
Hemophilia C
- AKA Rosenthal syndrome
- autosomal inheritance; can be dom or rec depending on gene mutation
- more common in Ashkenazi Jewish population
- symptoms do not correlate with FXI deficiency (not linear)
> usually mild bleeding
> lab monitoring not useful - treatment = plasma transfusions or FXI concentrates
T or F. Acquired coagulation disorders are more common than inherited disorders
T!
usually involve multiple coagulation factor deficiencies
Fibrinogen disorders
- Afibrinogenemia
- Hypofribrinogenemia
- Dysfibrinogenemia
Afibrinogenemia
- rare; severe fibrinogen def or absence of fibrinogen
- severe bleeding after slight trauma/poor wound healing, hemarthroses (bleeding into joints)
hypofibrinogenemia
- fibrinogenbelow ref range (<1.0g/L)
- bleeding mild to moderate
dysfibrinogenemia
- alteration in fibrinogen structure leading to nonfunctional molecule
- variable bleeding
- variable coag results
treatment for fibrinogen disorders
fibrinogen concentrate = prophylaxis, prior to surgical procedures, after trauma
blood products: plasma or cryoprecipitate; for active bleeds
factor XIII deficiency
- AKA fibrin stabilizing factor
- poor wound healing (initial clot formed but starts bleeding again)
- bleeding from umbilical cord stump
- bleeding into soft tissues
- bruising
- CNS bleeding
- recurrent spontaneous abortion
FXIII deficiency treatment
FXIII concentrate
contact factors deficiencies
- no bleeding, but thrombosis is possible with FXII deficiency
> role in activation of fibrinolysis - aPTT prolonged
- PT normal
- not required for in vivo coagulation (XII, PK, HMWK)
causes of acquired coag disorders
- abnormal or inadequate production (vit K deficiency, liver disease)
- increased use and or destruction (DIC, fibrinolysis)
- pathological inhibitors
- others = renal failure, acute trauma
sources of vitamin K
- exogenous = diet (leafy greens)
- endogenous = synthesis by gut flora
causes of factor deficiencies (vit K def)
- premature infant
- hospitalization and parenteral therapy
- malabsorption
- drugs: antibiotics, warfarin
- congenital vitamin K deficiency
what is vitamin K’s role in coagulation?
gamma-carboxylation of II, VII, IX, X (not all serine proteases)
diagnosis for vit K deficiency and treatment
aPTT and PT prolonged; supplements (oral or IV)
thrombopoietin
Thrombopoietin is a glycoprotein hormone produced by the liver and kidney which regulates the production of platelets. It stimulates the production and differentiation of megakaryocytes, the bone marrow cells that bud off large numbers of platelets
liver disease results in multiple hemostatic abnormalities
- decreased platelets: decreased thrombopoietin; splenic sequestration
- inadequate production of several coag factors
- inadequate production of regulatory proteins = antiplasmins, antithrombin, plasminogen, protein C and S
- abnormal factors (dysfibrinogenemia)
- defective clearance of hemostatic debris
- may result in DIC
