section 9 - factor deficiencies Flashcards
1
Q
factor VIII:C deficiency
A
hemophilia A
- most common deficiency in USA
- X linked recessive
- deficiency in vWF:VIII complex
- causes clinical bleeding
2
Q
Factor IX deficiency
A
- hemophilia B
- 2nd most USA common
- X linked recessive
- less severe than A
3
Q
acquired VIII deficiency
A
- DIC: being used up
- Liver disease: not using enough
4
Q
acquired IX deficiency
A
- DIC
- liver disease
- vit K deficiency (IX is vit K deficient)
- oral anti coagulants
5
Q
list fibrinogen deficiencies
A
- afibrinogenemia: hereditary = bleeding
- hypofibrinogenemia: <100mg/dL = bleeding
- dysfibrinogenemia: abnormal functionality = bleeding & thrombosis
6
Q
list the differential diagnosis of dysfibrinogenemia
A
- fibrinogen activity: low
- fibrinogen antigen: increased
7
Q
acquired fibrinogen deficiencies
A
- DIC
- fibrinogenolysis
- liver disease
8
Q
describe hyperfibrinogenemia
A
- fibrinogen = acute phase reactant
- physiologic stress causes fibrinogen increase
- can lead to thrombosis
9
Q
describe prothrombin deficiency
A
- rarest of congenital deficiencies
- autosomal recessive
- heterozygotes = asymptomatic
- homozygotes = bleeding
10
Q
acquired prothrombin deficiency
A
- DIC
- liver disease
- vitamin K deficiecny
- oral anticoagulants
11
Q
describe factor V deficiency
A
- autosomal recessive (both sexes impacted equally)
- only homozygotes have symptomatic bleeding
- treatment with FFP not cryo
12
Q
acquired factor V deficiecny
A
- DIC
- liver disease
13
Q
describe factor VII deficiency
A
- autosomal recessive
- 1/500,000 cases
- only homozygotes have symptoms
- treat with FFP, prothrombin complex concentrate (PCC) and vit K supplements
14
Q
acquired factor VII deficiency
A
- DIC
- liver disease
- vitamin K deficiency
- oral anticoagulants
15
Q
factor X deficiency
A
- autosomal recessive
- heterozygotes = mild bleeding
- homozygotes = severe bleeding
