Disorders of secondary hemostasis

Question 1

von Willebrand’s disease

Answer 1

Absent or defective vWF, abormal PLT adhesion to collagen, also carries factor VIII
PFA: inc. PT: N APTT: inc (classic type), 1:1 mix: corrects

Question 2

Treatment for vWF disease

Answer 2

DDAVP, FVIII concentrates

Question 3

Hemophilia A

Answer 3

X-linked recessive, Spontaneous bleeding in early childhood, defective VIII
PFA: N PT: N APTT: inc, 1:1 mix: corrects

Question 4

Hemophilia B

Answer 4

sex-linked recessive, sponetaneous bleeding with mild trauma, factor IX deficiency
PFA: N PT: N APTT: inc. 1:1 mix: corrects

Question 5

Prekallikrein (fletcher factor) deficiency

Answer 5

Autosomal recessive, no bleeding problems, but problems with thrombosis
PFA: N PT: N APTT: Inc

Question 6

Facotor XII (Hageman) deficiency

Answer 6

No bleeding, thrombotic disease
PFA: N PT: N APTT: inc

Question 7

Hemophilia C

Answer 7

Deficiency of XI/PTA, autosomal recessive, delayed mild bleed plus post pre-op, Ashkenazi Jew population

Question 8

Hereditary Afibrinogemia

Answer 8

Marked decrease in fibrinogen production, autosomal recessive, Severe bleeding at birth (not as severe as hemophilia A)
PFA: N PT: inc. APTT: inc. Prolonged thrombin time

Question 9

Dysfibrinogenemia

Answer 9

Abnormal functioning fibrinogen molecule, Plasmin cannot degrade, may cause bleeding or thrombosis
Prolonged thrombin time

Question 10

Factor XIII deficency

Answer 10

Autosomal recessive, Bleeding from umbilical cord, spontaneous abortion, cerebral bleed
Abormal 5 M urea solubility test only

Question 11

Disseminated intravascular coagulation

Answer 11

uncontrolled formation of fibrin. Clotting proteins are consumed faster than produced, leads to bleeding. Widespread lysing of the clots. uncontrolled thrombin and plasmin circulate. =Severe bleeding and vessel occlusion

Question 12

What triggers DIC?

Answer 12

OB complications, Acute promyelocytic leukemia, Gram-negative septicemia,

Question 13

Lab results of DIC

Answer 13

Schisotcytes (sheared through clots), prolonged PT and APTT, decreased fibrinogen, positive D-dimers and FDPs, thrombocytopenia, Prolonged thrombin time

Question 14

Vitamin K deficency

Answer 14

Severe liver disease (also newborns), abnormal vitamin K dependent factors: II, VII, IX, X
PT: inc. APPT: maybe inc.

Question 15

Circulating inhibitors

Answer 15

often autoantibodies, 1:1 mix does not correct prolonged screening tests

Question 16

Inhibitors to factor VIII

Answer 16

often in patients with hemophilia A after treatment, multiple myeloma and waldenstroms, prolonged APTT

Question 17

Inhibitors to Factor IX

Answer 17

Seen in patients with hemophilia B after therapy, prolonged APPT

Question 18

Lupus anticoagulant

Answer 18

Commonly directed against plt phospholipid, associated with SLE but not always, prolonged APTT
Associated with THROMBUS and spontaneous abortions

Question 19

Prevalence of causes of uncorrected mixing study

Answer 19

1: Heparin
2: Lupus inhibitor
3: VIII inhibitor
4: IX inhibitor

Question 20

Factor V Lieden

Answer 20

most common hereditary thromus disorder, mutation of Factor V causing protein C resistence

Question 21

Factor II 20210 mutation

Answer 21

second leading hereditary cause of thrombus, genetic mutation of factor II

Question 22

Antithrombin defiency

Answer 22

lack of inhibitor promotes clotting

Question 23

Deficiency of factor XII

Answer 23

Decreased activation of fibrinolysis, causing thrombus