Blood wk 2, clinical Flashcards
what does a deficiency in factor 8 cause?
Hemophilia A
who is more commonly effected by hemophilia A?
males (on X chromosome)
what is the difference between mild, moderate and severe hemophilia?
mild: 49%
moderate: 6-49%
severe: less than one (could severely bleed at any time)
which is more likely hemophilia A or B?
A is 4x more common
what occurs with hemophilia A?
most people only experience issues after major bleeding event like tooth extraction or surgery
what are the genetics of hemophilia?
X inked recessive
(effected males will pass to a daughters as carriers, not sons)
30% cases spontaneous
what is the treatment for hemophilia A?
DDAVP- mild cases
Factor 8 replacements
what is the newest treatment for hemophilia?
roctavian treatment gene therapy (using virus vectors)
what causes hemophilia B?
factor IX
what is the treatment for hemophilia B?
routine treatment regimen of intravenous infusions of Factor IX
replacement products to maintain sufficient levels of clotting factor to prevent bleeding episodes
what is the treatments for bleeding patients with vit K deficiency?
fresh frozen plasma (also subcutaneous vit k)
what is hemgenix?
one-time gene therapy product given as a single dose by IV infusion
what do you do for a patients with supratherapeutic INR values due to being on warfarin and an antibiotic?
1- make sure not bIeeding
2-INR is >5, warfarin
should be withheld for at least 1 dose, and close follow-up
monitoring should be arranged
what is something to be aware of for patients taking warfarin?
need to keep a constant diet, too much K can throw everything off
what s the cheapest simplest thing to do for someone bIeeding and need of quick correction?
fresh frozen pIasma
what is the mechanism of warfarin?
vit K epoxide reductase inhibitor (keeps kit K reduced)
what is the site f action for heparin?
anti-thrombin III
what does heparin do?
reduction in activity and synthsis of thrombin
can you use heparin or warfarin during pregnancy?
heparin yes
warfarin no
what is von wiIIiebrand disease?
vWF stored in Weibel-Palade bodies of endothelial cells & alpha granules of megakaryocytes/platelets
what is the genetic inheridance of von wiIIiebrand?
autosomaI dominant
why is there a cIotting issue seen with VWB?
because the first step of co-ag cant happen, pIateIets can not ahere to the waII
what is thrombocytopenia?
low platelet count (beow 150,000)
what are the major pathophysiologic mechanisms of
thrombocytopenia?
- Decreased platelet production in the bone marrow
- Peripheral platelet destruction by antibodies
- Consumption in thrombi
- Sequestration (pooling) of platelets in the spleen in individuals with portal hypertension and/or splenomegaly