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REBEL Review 79: Treatment of Hemophilia 

Walkina Out of Pt Room, You Should Know... 
1. Type
Factor Activity ... Type of Factor ... ) Units of Factor ... #factor9 #factorviii ... #factorix
Differential Diagnosis for a Prolonged PT and aPTT
If the PT and the aPTT are both prolonged,
could be multiple factors ... pathways or a single factor ... common pathway factors ... Decreased factor ... Factor consumption
Hemostasis Physiology

The blood's response to blood vessel injury can be viewed as four interrelated steps. 
Roman
to coagulation factors ... PF-3, platelet factor
Posterior Reversible Encephalopathy Syndrome (PRES) - Diagnosis
Clinical Features:
 • Altered mental status (Either lethargy, confusion, or
PRES) - Diagnosis Clinical ... blindness) Risk Factors ... Diagnosis: Risk Factor ... + Clinical Features
Mixing Study
 • Clotting tests can be abnormal due to either a clotting factor deficiency, or
either a clotting factor ... inhibitor of normal factor ... generally only require factors ... Patient has a factor ... Patient has a factor
Bleeding and Bruising - Disorders of Hemostasis - Differential Diagnosis Framework

== Quantitative or Qualitative defect in
a coagulation factor ... Von Willebrand factor ... Von Willebrand factor ... inhibitors - factor ... V, or factor X
Blood Product Components, Contents, Volume and Shelf Life

Whole blood (autologous or directed donations) - RBC and
Factors V and VIII ... all coagulation factors ... least 80 units of factor ... von Willebrand factor ... , factor XIII, fibronectin
Hemophilia - Diagnosis and Management
Diagnosis:
 • Consistent bleeding history (unless screening for disease in family members
von Willebrand factor ... normal Individual factor ... with relevant factor ... Serious: 100% factor ... Moderate: 50% factor
On the INR of FFP - why transfusing for INR < 1.7 is futile

FFP replaces a
of coagulation factors ... 30% coagulation factors ... fixed increase in factor
Causes of Abnormal PT and/or aPTT
Prolonged Prothrombin Time (PT)
1. Acquired deficiency of FVII
Contact pathway factors ... inhibitors to one factor ... Decreased factor ... Factor consumption ... Factor X deficiency