Section 9 Disorders of 2nd hemostasis factor defficiencies

Question 1

Factor VIII:C deficiency

Answer 1

Hemophilia A
Most common
X link recessive

Question 2

Hemophilia A lab findings

Answer 2

Clinical Bleeding
PFA: normal
PT: normal
APTT: prolonged (intrinsic pthwy
Confirmed with factor VIII assay

Question 3

Aquired VIII deficiency

Answer 3

DIC
Liver disease

Question 4

Factor IX deficiency

Answer 4

Hemophilia B
Royal disease
X-linked, almost exclusively males

Question 5

Hemophilia B lab findings

Answer 5

Clinical bleeding
PFA: normal
PT: normal
APTT: Prolonged
Confirm w/ factor IX assay

Question 6

Aquired Factor IX deficiency

Answer 6

DIC
Liver disease
Vit K
Oral anticoags: warfarin,Coumadin

Question 7

Fibrin deficiencies

Answer 7

Afibrinogenemia: low or no fibrinogon. Bleeding
Hypofribrinogenemia: <100 mg/dl. Bleeding
Dysfibrinogenemia: both bleeding and thrombosis possible

Question 8

Hyperfibrinogenemia

Answer 8

Fibrin = acute phase reactant
stress causes increase
leads to thrombosis

Question 9

factor II deficiency

Answer 9

Prothrombin
Rarest of the congenital deficiencies
autosomal recessive

Question 10

Prothrombin deficiency symptoms

Answer 10

Homozygotes: bleeding

Question 11

Factor V deficiency

Answer 11

1:1mil
Autosomal recessive
homozygotes: symptomatic bleeding

Question 12

Factor VII deficiency

Answer 12

1:500k population

Question 13

Factor X deficiency

Answer 13

1:500k population
Heterozygotes can have mild bleeding (unique property)
Acquired all standard, except Amyloidosis: starch like substance on organs

Question 14

Factor XI deficiency

Answer 14

Hemophilia C
1:100k
ONLY CONTACT FACTOR DEFICIENCY THAT RESULTS IN BLEEDING
Decreased production of TAFI

Question 15

Factor XII deficiency

Answer 15

APTT increase but no bleeding:
Impact plasmin production ->thrombosis

Question 16

PK deficiency

Answer 16

Rare
APTT increase -> no bleeding->thrombosis

Question 17

HMWK deficiency

Answer 17

Asymptomatic
Thrombosis

Question 18

Factor XIII deficiency

Answer 18

Very rare
Symptoms: umbilical stump bleeding, poor would healing, bruises resolve slowly, easy scar formation

Question 19

DIC

Answer 19

Over stimulation of coag cascade

Question 20

DIC
Fibrinolysis vs. fibrogenolysis

Answer 20

Fibrinolysis: D-Dimer increase, plt count decrease, schistocytes seen
Fibrogenolysis: D-dimer/plt count/blood smear: normal

Question 21

DIC treatment

Answer 21

Remove the cause
Give heparin

Question 22

Liver disease

Answer 22

Deficiency of factors and inhibitors
dysfunctional factors & inhibitors
Results in bleeding or thrombosis

Question 23

Vit K deficiency

Answer 23

effects factors II,VII,IX, X
Protein C

Question 24

Mass transfusions

Answer 24

storage of labile factors V&VIII
Citrate toxicity (give Ca)
Thrombocytopenia