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HandL Final > Disorders Of Clotting And Platelets - Hubbard > Flashcards

Disorders Of Clotting And Platelets - Hubbard Flashcards

1
Q

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

A

Antiphospholipid syndrome

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2
Q

What does a hypercoagubility panel include?

A

Factor V Leiden
Prothrombin gene mutation
Protein C and S

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3
Q

Major CFs of pts with thrombocytopenia?

A 
Petechiae
Ecchymoses 
Purpura
Epistaxis
GI, GU bleeding
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4
Q

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

What tx for pregnant bitches w/APS?

A

Hydroxychloroquine

SC heparin

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5
Q

What is generally required for diagnosis of antiphospholipid syndrome?

Treatment?

A

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

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

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6
Q

Schistocytes aka helmet cells seen in what?

A

Microangiopathic anemia

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

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7
Q

Coagulation factor deficiency CFs?

A

Hemarthroses
Deep hematomas
Delayed bleeding after surgery/trauma

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8
Q

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

W/renal failure?

A

TTP

HUS

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9
Q

Tx for vWF disease?

A

Cryoprecipitate replaces vWF

DDAVP (desmopressin) causes release of vWF from endothelium

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10
Q

What can cause coagulopathy?

A

Severe liver disease

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11
Q

What can Lupus anticoagulant cause?

A

Prolonged PTT

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12
Q

Increased PT seen in pts taking what?

Normal time?

A

Warfarin or coumadin

10-13s

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13
Q

50% of pts w/ATIII def have what?

A

DVT and/or PE by age 30

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14
Q

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

Explain

A

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

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15
Q

Describe the HELLP syndrome

Seen in whom?

A

Hemolytica anemia, elevated liver enzymes, low platelets

Seen in obstetric pts, tx is deliver baby

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16
Q

What agent suppresses the white count?

A

Viral illness –> EBV ie mono

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17
Q

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

A

Ristocetin

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18
Q

What is the most common cause of bleeding?

A

Thrombocytopenia

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19
Q

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

A

AT-III deficiency

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20
Q

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

A

Osler-Weber-Reneau

aka Hereditary hemorrhagic telangiectasia

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21
Q

Mild thrombocytopenia with GIANT platelets known as what?

A

Bernard-Souiller syndrome

22
Q

What are the 3 tests used to diagnose antiphospholipid syndrome?

A

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

23
Q

Common sx in thrombocytopenia?

A

Petechiae (pinpoint bleeds)

Purpura

24
Q

How do you diagnose AT III deficiency?

Treatment?

A

Level in serum, < 50% of normal

Prophylactic tx w/anticoagulants
Need more heparin

25
Q

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

A

Warfarin

26
Q

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

A

Gray platelet syndrome

27
Q

Agranular platelets is what disease?

A

Gray platelet syndrome

28
Q

What is Mild Hemophilia A?

Moderate?

Severe?

A

6-25% normal activity

1-5

< 1

29
Q

Associated features in antiphospholipid syndrome?

A

CT disease
Inc PTT
Valvular HD
CAD

30
Q

1st line tx for ET?

2nd?

A

Hydroxyurea

Anagrelide

31
Q

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

Result?

A

Prothrombin 20210

Hypercoaguable state

32
Q

Tx for Factor V Leiden w/no prior episodes?

W/prior episodes?

A

Observation Duh, DVT prophylaxis for surgery

Lifelong anticoagulation therapy

33
Q

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

A

Glanzmann

34
Q

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

Why?

A

Observation

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

35
Q

Hereditary hemorrhagic telangiectasia inheritance?

Defect in what gene?
Chromosome?

A

AD

Endoglin (CD 105)
9

36
Q

Hereditary hemorrhagic telangiectasia clinical course?

Treatment?

A

Usually benign

Surgery and laser photoablation

37
Q

What causes acral erythema?

What is it?

A

Ara-C

Tips of toes, fingers, penis get red

38
Q

What syndrome are the following CFs describing?

Easy bleeding and bruisability
Hematomas from bleeding into soft tissues
Hemarthroses
Bleeding after surgery

A

Hemophilia A

39
Q

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

A

Bernard-Soulier syndrome

Impaired aggregation to ristocetin also present in vWF

40
Q

Erythromelalgia is a vasomotor change that occurs in pts with what?

What may accompany?

Treatment?

A

ET

Burning pain

baby ASA

41
Q

What is the life saving tx in TTP?

A

Plasmapharesis

42
Q

What is the most common location for routine epistaxis?

A

Kesselbach’s plexus

43
Q

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

A

ITP

44
Q

Bleeding from every orifice is indicative of what?

Treatment?

A

DIC

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

45
Q

What sx associated w/viral infection?

A

Suppressed wbc count
Arthralgia/myalgia
Fever
Splenomegaly

46
Q

ADAMTS13 is known as what?

A

vWF cleaving protease

47
Q

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

Why?

A

Warfarin use

Starting warfarin results in temporary increase in coaguability

48
Q

Hereditary TTP due to what?

Acquired?

A

Mutation of ADAMTS13

Autoantibodies directed at ADAMTS13

49
Q

PTT prolonged in pts taking what?

Normal time?

A

Heparin

25-40s

50
Q

What can cause a false-positive VDRL?

A

Anticardiolipin syndrome

HandL Final (8 decks)

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