coagulopathies

Question 1

name some hereditary primary bleeding disorders

Answer 1

• vWD
• qualitative platelet disorders (storage pool, Glanzmann, Bernard-Soulier)
• congenital thrombocytopenia

Question 2

name some acquired primary bleeding disorders

Answer 2

Medications
Uremia
Thrombocytopenia
Immune
Non-immune
Cardiac bypass
Myeloproliferative d/o
Acquired vWD

Question 3

name some hereditary secondary bleeding disorders

Answer 3

Hemophilia
Rare coagulation factor deficiencies
Fibrinolytic defects

Question 4

name some acquired secondary bleeding disorders

Answer 4

Liver disease
DIC
Vitamin K deficiency
Acquired coagulation factor inhibitors
Anticoagulation

Question 5

acquired vessel wall bleeding disorders

Answer 5

• vitamin C deficiency

- vascular trauma

Question 6

vWD inheritance pattern

Answer 6

autosomal dominant

Question 7

two functions of vWF

Answer 7

• tethers platelets to collagen

- chaperones factor VIII

Question 8

quantitative types of vWD

Answer 8

1 and 3

Question 9

qualitative types of vWD

Answer 9

2

Question 10

lab tests for vWD

Answer 10

coagulations screening

• PT and TCT normal
• aPTT possibly prolonged if factor VIII low
• VIII low

platelet screening

• prolonged PFA-100
• prolonged bleeding time
• abnormal aggregation with ristocetin

Question 11

treatment for vWD

Answer 11

• DDAVP (for type 1 and some type 2)
• vWF replacement (cryoprecipitate, alphate)
• antifibrinolytic therapy
• birth control for menorrhagia

Question 12

what is DDAVP and what does it do?

Answer 12

desmopressin, synthetic vasopressin

stimulates release of vWF and VIII from endothelial cells

Question 13

when avoid DDAVP in treatment of vWD?

Answer 13

type 2B, exacerbates bleeding

Question 14

deficiency in hemo A?

Answer 14

VIII

Question 15

deficiency in hemo B?

Answer 15

IX

Question 16

inheritance patterns of hemophilia

Answer 16

• A and B are x-linked recessive

- C, parahemophilia are autosomal recessive

Question 17

deficiency of hemo C?

Answer 17

XI

Question 18

deficiency of parahemophilia?

Answer 18

V

Question 19

what is common in the female carrier state of hemophilia?

Answer 19

menorrhagia

Question 20

what are considered mild, moderate and severe for hemophilia A and B?

Answer 20

mild = 5-40% of factor level
moderate = 1-5% of factor level
severe = <1% of factor level
normal factor level is 50-150%

Question 21

expected labs in hemophilia A and B

Answer 21

• platelet function normal
• PT normal
• prolonged aPTT except possibly in mild
• aPTT will correct with mixing study
• low specific factor levels

Question 22

bleeding manifestations in hemophilia?

Answer 22

• may be delayed because primary still happens
• bleeding into joints
• CNS
• bleeding after minor trauma
• hematuria

Question 23

pathological issue with hemophilia

Answer 23

no thrombin burst with no IX or VIII

Question 24

hemophilia treatment

Answer 24

• recombinant IX and VIII, replacing plasma derived
• DDAVP sometimes effective in mild hemophilia
• factors can be used prophylactically in severe hemophiliacs to keep factor level above 1%

Question 25

complications of hemophilia

Answer 25

- chronic arthropathy - infectious diseases - development of antibodies against missing factor (might have to bypass using VIIa)

Question 26

explain acquired hemophilia

Answer 26

- spontaneous autoantibody to factor VIII | - most are idiopathic

Question 27

labs in acquired hemophilia

Answer 27

- platelets and PT normal - aPTT prolonged - aPTT will not correct with mixing study - low factor VIII

Question 28

treatment of acquired hemophilia

Answer 28

- low antibody titer, treat with factor VIII - high antibody titer, treat with bypass agent (recombinant VII or FEIBA which includes II, VII, IX, X) - eradication by immune suppression

Question 29

characteristics of fibrinolytic bleeding

Answer 29

- delayed 12-24 hours - mucocutaneous/oropharyngeal - menorrhagia, GU

Question 30

is hereditary or acquired fibrinolytic bleeding more common?

Answer 30

acquired

Question 31

explain hereditary fibrinolytic bleeding

Answer 31

- autosomal recessive - deficiency in alpha-2-antiplasmin or PAI-1, leaving more plasmin to break down fibrin - over expression of plasminogen activator (Quebec platelet disorder)

Question 32

labs in fibrinolytic bleeding

Answer 32

- PT and PTT may be prolonged because fibrin is being broken down - hard to distinguish from DIC - increased products of fibrin breakdown (d-dimers)

Question 33

treatment for fibrinolytic bleeding

Answer 33

- plasma infusions - anti-fibrinolytic drugs (if no DIC) - Amicar - tranexamic acid

Question 34

what is the most common cause of bleeding due to vitamin K deficiency?

Answer 34

over anticoagulation with warfarin

Question 35

causes of vitamin K deficiency

Answer 35

- dietary (malabsorption, alcoholism) - medications - coumarins (warfarin)

Question 36

labs in vitamin K deficiency

Answer 36

- PT and aPTT prolonged (PT only in early deficiency) - PT and aPTT will correct with mixing study - platelets normal - decreased II, VII, IX, X - FACTOR V NORMAL!

Question 37

treatment of vitamin K deficiency

Answer 37

- oral or IV vitamin K (corrects in hours) - fresh frozen plasm (only in emergencies, corrects factors immediately, must be used with vitamin K) - prothrombin complex concentrates (II, VII, IX, X)

Question 38

what is preserved in liver failure?

Answer 38

factor VIII

Question 39

what does liver disease look like for bleeding?

Answer 39

- both bleeders and clotters, but bleeding usually wins out

Question 40

lab findings in coagulopathy of liver disease

Answer 40

- prolonged PT/INR and aPTT - low factors in INCLUDING V, but VIII is still preserved - PT/INR is unreliable

Question 41

liver disease treatment

Answer 41

- transplant | - vitamin K and fresh frozen plasma can be temporary fixes

Question 42

DIC - major relevant pathology for bleeding

Answer 42

- excessive thrombin, increases fibrin and activates platelets - compensatory increase in APC, plasmin, and fibrinolysis - increased activation of endothelium, more TF, more coagulation

Question 43

labs in DIC

Answer 43

- prolonged PT/INR and aPTT - elevated d-dimers - low platelets - low fibrinogen - anemia - RBC schistocytes - low AT, APC, S

Question 44

treatment for DIC

Answer 44

- treat underlying cause - transfusion support plasma (coagulation factors) cryoprecipitate (fibrinogen) platelets/RBC - heparin (only in active thrombosis)