Disorders Of Clotting And Platelets - Hubbard

Question 1

What has the features of thromboembolic phenomena, miscarriage, thrombocytopenia, cerebral ischemia and recurrent stroke?

Answer 1

Antiphospholipid syndrome

Question 2

What does a hypercoagubility panel include?

Answer 2

Factor V Leiden
Prothrombin gene mutation
Protein C and S

Question 3

Major CFs of pts with thrombocytopenia?

Answer 3

Petechiae
Ecchymoses 
Purpura
Epistaxis
GI, GU bleeding

Question 4

What medicine may be used to reduce thromboembolism in pts w/antiphospholipid syndrome and SLE?

What tx for pregnant bitches w/APS?

Answer 4

Hydroxychloroquine

SC heparin

Question 5

What is generally required for diagnosis of antiphospholipid syndrome?

Treatment?

Answer 5

Multiple (+) tests over a 3-12 month period

No tx for those w/out history, those w/hx need lifelong anticoag

Question 6

Schistocytes aka helmet cells seen in what?

Answer 6

Microangiopathic anemia

Think of the Schwartz and the micro actor who played Dark Helmet

Question 7

Coagulation factor deficiency CFs?

Answer 7

Hemarthroses
Deep hematomas
Delayed bleeding after surgery/trauma

Question 8

Microangiopathic hemolytic anemia, thrombocytopenia, fever, and neurologic symptoms characteristic of what?

W/renal failure?

Answer 8

TTP

HUS

Question 9

Tx for vWF disease?

Answer 9

Cryoprecipitate replaces vWF

DDAVP (desmopressin) causes release of vWF from endothelium

Question 10

What can cause coagulopathy?

Answer 10

Severe liver disease

Question 11

What can Lupus anticoagulant cause?

Answer 11

Prolonged PTT

Question 12

Increased PT seen in pts taking what?

Normal time?

Answer 12

Warfarin or coumadin

10-13s

Question 13

50% of pts w/ATIII def have what?

Answer 13

DVT and/or PE by age 30

Question 14

On your work up for thrombocytopenia what must you always exclude?

Explain

Answer 14

Pseudothrombocytopenia on peripheral blood smear

Platelets may clump together in part of EDTA tube and not be examined accurately, repeated in heparin or citrate tube

Question 15

Describe the HELLP syndrome

Seen in whom?

Answer 15

Hemolytica anemia, elevated liver enzymes, low platelets

Seen in obstetric pts, tx is deliver baby

Question 16

What agent suppresses the white count?

Answer 16

Viral illness –> EBV ie mono

Question 17

An abnormal result from what platelet aggregation test is the best way to assess a vWF disease?

Answer 17

Ristocetin

Question 18

What is the most common cause of bleeding?

Answer 18

Thrombocytopenia

Question 19

What condition presents with recurrent LE thrombophlebitis and DVT, venous insufficiency, and chronic leg ulcers?

Answer 19

AT-III deficiency

Question 20

What is the only endothelial syndrome associated w/hemostatc complications which also shows AV malformations and aneurysmal dilatations throughout the body?

Answer 20

Osler-Weber-Reneau

aka Hereditary hemorrhagic telangiectasia

Question 21

Mild thrombocytopenia with GIANT platelets known as what?

Answer 21

Bernard-Souiller syndrome

Question 22

What are the 3 tests used to diagnose antiphospholipid syndrome?

Answer 22

Increased PTT
Lack of correction in mixing studies
Neutralization of inhibitor w/excess phospholipid (DRVVT)

Question 23

Common sx in thrombocytopenia?

Answer 23

Petechiae (pinpoint bleeds)

Purpura

Question 24

How do you diagnose AT III deficiency?

Treatment?

Answer 24

Level in serum, < 50% of normal

Prophylactic tx w/anticoagulants
Need more heparin

Question 25

What is recommended in pts that have Protein C, S, or AT-III def?

Answer 25

Warfarin

Question 26

What is an ASA like defect w/impaired 2nd wave aggregation?

Answer 26

Gray platelet syndrome

Question 27

Agranular platelets is what disease?

Answer 27

Gray platelet syndrome

Question 28

What is Mild Hemophilia A? Moderate? Severe?

Answer 28

6-25% normal activity 1-5 < 1

Question 29

Associated features in antiphospholipid syndrome?

Answer 29

CT disease Inc PTT Valvular HD CAD

Question 30

1st line tx for ET? 2nd?

Answer 30

Hydroxyurea Anagrelide

Question 31

What is a mutation resulting in increased activity for prothrombin and inability to de-activate prothrombin? Result?

Answer 31

Prothrombin 20210 Hypercoaguable state

Question 32

Tx for Factor V Leiden w/no prior episodes? W/prior episodes?

Answer 32

Observation Duh, DVT prophylaxis for surgery Lifelong anticoagulation therapy

Question 33

Normal aggregation to ristocetin but everything else is abnormal is what disease?

Answer 33

Glanzmann

Question 34

If a pt has a platelet count of 41,000 with petechiae, what is the best tx? Why?

Answer 34

Observation No need to treat if platelet count is above 30,000

Question 35

Hereditary hemorrhagic telangiectasia inheritance? Defect in what gene? Chromosome?

Answer 35

AD Endoglin (CD 105) 9

Question 36

Hereditary hemorrhagic telangiectasia clinical course? Treatment?

Answer 36

Usually benign Surgery and laser photoablation

Question 37

What causes acral erythema? What is it?

Answer 37

Ara-C Tips of toes, fingers, penis get red

Question 38

What syndrome are the following CFs describing? Easy bleeding and bruisability Hematomas from bleeding into soft tissues Hemarthroses Bleeding after surgery

Answer 38

Hemophilia A

Question 39

Absent aggregation to ristocetin but normal aggregation to everything else is what disease?

Answer 39

Bernard-Soulier syndrome Impaired aggregation to ristocetin also present in vWF

Question 40

Erythromelalgia is a vasomotor change that occurs in pts with what? What may accompany? Treatment?

Answer 40

ET Burning pain baby ASA

Question 41

What is the life saving tx in TTP?

Answer 41

Plasmapharesis

Question 42

What is the most common location for routine epistaxis?

Answer 42

Kesselbach's plexus

Question 43

What is a possible manifestation related to a uri, ct disease, CLL, or indolent lymphoma, especially in a young adult?

Answer 43

ITP

Question 44

Bleeding from every orifice is indicative of what? Treatment?

Answer 44

DIC Treat underlying cause and supportive care w/platelet and factor replacement

Question 45

What sx associated w/viral infection?

Answer 45

Suppressed wbc count Arthralgia/myalgia Fever Splenomegaly

Question 46

ADAMTS13 is known as what?

Answer 46

vWF cleaving protease

Question 47

What is the most common cause of hypercoagulable state from protein C and S deficiency? Why?

Answer 47

Warfarin use Starting warfarin results in temporary increase in coaguability

Question 48

Hereditary TTP due to what? Acquired?

Answer 48

Mutation of ADAMTS13 Autoantibodies directed at ADAMTS13

Question 49

PTT prolonged in pts taking what? Normal time?

Answer 49

Heparin 25-40s

Question 50

What can cause a false-positive VDRL?

Answer 50

Anticardiolipin syndrome