Bleeding Disorders Flashcards
1
Q
Causes
A
- platelet dysfunction
- coagulation factor disorder
2
Q
Categories
A
- primary hemostasis (platelet and endothelial disorders (thrombocytopenia and vWD))
- secondary (coagulation factor deficiency including Hemophilia A and B)
3
Q
Evaluation (history)
A
- history (duration, frequency, timing)
- source of bleeding
- response to procedures or trauma
- fam hx or known genetic bleeding disorder
- review of medications
4
Q
Evaluation (physician exam)
A
- signs of ongoing bleeding including internal bleeding
- mucosal bleeding, petechiae
- splenomegaly or hepatomegaly
5
Q
Investigations
A
- CBC
- PBS
- PT (measure activity of warfarin and vit K antagonists), aPTT (factor VII, IX, or XI deficiencies in patients w/ normal PT and prolonged aPTT)
- bleeding time
6
Q
vWD
A
- most common inherited bleeding disorder
- autosomal inheritance pattern
- VWF needed for platelet adherence to endothelium
- in absence, mild-mod mucocutaneoux bleeding
- 3 types correspond to type of dysfunction
7
Q
Hemophilia A and B
A
X-linked congenital bleeding disorder caused by deficiency of coag factor VII or factor IX
8
Q
Hemophilia A&B lab testing
A
- normal PT with prolonged PTT that corrects with a 50/50 mixing study.
- level of disease severity depends on the amount of clotting factor:
- severe: <1% of normal level (spontaneous bleeding into muscles or joints)
- moderate: 1%–5% of normal level (occasional spontaneous bleeding; prolonged bleeding with minor trauma or surgery)
- mild: 5%–<40% of normal level (spontaneous bleeding is rare; severe bleeding with trauma or surgery)
9
Q
Hemophilia A/B management
A
- replacement with FVIII and FIX concentrates
- be aware that patients an develop inhibitors that inactivate concentrates
(might need activated F VII to treat) - also use desmopressin (increases factor VIII levels) - used for mild hemophilia A, mild bleeding, minor invasive procedures
