Bleeding Disorders Flashcards
Hemophilias
hemophilia A - factor 8 (VIII) deficiency
-MC*
hemophilia B - factor 9 (IX) deficiency
-Christmas disease
congenital clotting disorders
- X-linked recessive (so–>affects males)
- Severe pain in weight-bearing joint (hemophilic arthropathy)*
Labs: Instrinsic pathway affected so aPTT is long. Bleeding time is normal in hemophilia
Platelet disorders
- Dysfunction (acquired or congenital)
- Splenic spequestration
- Increased destruction
- Impaired production
Signs of bleeding
Petechiae (<2mm)
Purpura (2-10mm)
Ecchymosis (>1cm)
What are the 4 components of a basic screen for a patient you suspect has a bleeding disorder?
- CBC
- Peripheral smear
- PT/INR, aPTT
- Bleeding time
Platelet counts
Normal:
150,000 -450,000/mm3
Thrombocytopenia:
<100,000/mm3
When does spontaneous bleeding occur?
<20,000/mm3 platelets
Bleeding time
the measure of time from bleeding to hemostasis (coagulation)
Prolonged bleeding time:
- Platelet disorder (ex. von Willebrand’s Disease)
- Severe thrombocytopenia
What are the 3 stages of hemostasis
Just after vessel injury…
- Vasoconstriction
“vascular spasm”
-more effective in small vessels - PRIMARY Hemostasis/Platelet plug formation:
- activated by von Willebrand factor
- platelets adhere to exposed collagen, release ADP and thrombaxane A2 - SECONDARY Hemostasis/Coagulation cascade:
- formation of fibrin from fibrinogen
- Extrinsic, Instrinsic, and Common pathways
What do Protein C, S, and ATIII do?
regulate clot formation.
If you don’t have these, you clot TOO MUCH!
Prothrombin Time (PT)
- Extrinsic pathway (or common)
- For monitoring Warfarin
PTT or aPTT (Activated partial thromboplastin Time)
- Intrinsic pathway (or common)
- For monitoring heparin therapy (not LMWH)
-Significant for Hemophilia, vW disease, Vitamin K deficiency
INR (International Normalized Ratio)
-more accurate, standardized way of looking at PT (extrinsic pathway)
MC sites of bleeding for hemophilias A & B
- Joints (spontaneous hemarthrosis, is sign of severe disease)
- Muscles
- Skin
- GI
- GU
Hemophilia A: Labs
- Platelets and PT are normal
- aPTT will be prolonged**
Hemophilia A: treatment
- Factor VIII infusions
- Desmopressin
- AVOID aspirin