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Clin Med hematology > clotting disorders > Flashcards

clotting disorders Flashcards

1
Q

What is hemophilia A

A

Congenital factor 8 deficiency

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2
Q

What is the most common hemophilia

A

Hemophilia A

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3
Q

Which gender typically shows signs of hemophilia A

A

Males (X-linked disorder)

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4
Q

Is hemophilia a clotting disorder or a bleeding disorder

A

Clotting disorder

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5
Q

What is hemophilia B

A

Congenital 9 deficiency
*males mostly affected

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6
Q

What occurs in the body for hemophilia to be a clotting disorder

A

Lack of factor 8 or 9 disrupt the cascade which causes a weak platelet plug

= causes weak/delayed fibrin clot

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7
Q

What disease is also known as Christmas disease

A

hemophilia B

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8
Q

What is hemophilia C

A

Factor 11 deficiency

*not X-linked

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9
Q

What is a common clinical presentation of hemophilia

A

*mulitple episodes of prolonged bleeding
-hemarthrosis (ankles, knees, elbows)

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10
Q

What is a target joint

A

4 or more bleeds within 6 months

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11
Q

Which lab measures the intrinsic pathway

A

PTT

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12
Q

Which lab measures the extrinsic pathway

A

PT

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13
Q

What is DDAVP

A

Desmopressin

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14
Q

What is the purpose of giving DDAVP

A

Synthetic vasopressin to stimulate VWF production

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15
Q

When can DDAVP be used with hemophilia

A

Only for mild or moderate disease

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16
Q

If a patient develops factor 8 antibodies, what can you give them

A

factor 7

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17
Q

What is the most common inherited coagulation disorder

A

Von Willebrands disease (vWF on chromosome 12)

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18
Q

Which gender shows more symptoms of vWF

A

Females because of menstruation and pp bleeding

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19
Q

What is the role of vWF

A

Activate X–> Xa
Promotes platelet aggregation
Bind to factor 8 to extend its life

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20
Q

What is the most severe form of vWF disease

A

Undetectable (Type 3)

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21
Q

How does vWF present

A

Mucocutaneous bleeding
heavy menstruation

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22
Q

What is the definitive diagnosis for VWD

A

Looking at clotting factors

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23
Q

How do you treat type 1 VWD

A

DDAVP (desmopressin)

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24
Q

What causes a primary hyper coagulable state

A

Impairment in antithrombin, factor 10, and protein C and S

Prothrombin gene mutation

Anti- phospholipid antibody syndrome

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25
Q

What are some acquired / secondary causes of hyper coagulable States

A

Pregnancy
Malignancy
Surgery / trauma
Polycythemia vera

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26
Q

What are natural anticoagulants

A

Antithrombin
protein C&S

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27
Q

What is the role of protein C & S

A

Block factor 5 and 8

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28
Q

What is the role of antithrombin

A

block factor 10 and thrombin

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29
Q

how do hyper coagulable states present

A

DVT
PE
MI
Stroke
pregnancy loss

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30
Q

What is antiphospholipid antibody syndrome super relevant for

A

Pregnancy and pregnancy loss

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31
Q

What are some risk factors of antiphospholipid syndrome

A

Hep B
Hep C
Lyme

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32
Q

What is thrombocytopenia

A

Low platelet count

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33
Q

At what point will you see spontaneous bleeding in thrombocytopenia

A

10-20K

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34
Q

How does thrombocytopenia present

A

Excessive/repetitive bleeding
excessive bruising
Bleeding at weird sites
Excess mucosal bleeding
petechiae

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35
Q

How would you workup thrombocytopenia

A

CBC with platelet count
Peripheral smear
Platelet function analysis
CMP (kidney function)
D-dimer (concurrent thromboses)

36
Q

What are examples of concurrent thromboses with thrombocytopenia

A

DVT
HIT
DIC

37
Q

What is DIC classically associated with

A

Gram negative organisms

38
Q

What is the most common cause of DIC

A

Sepsis

39
Q

What is occurring with DIC

A

Coagulation and fibrinolysis pathways in overdrive

40
Q

What is consumption coagulopathy

A

Clots everywhere, leads to organ ischemia, uses up platelets and other clotting factors and other areas of the body start to bleed from minor injuries

41
Q

What is Acute DIC

A

Quick / larger trigger
-sepsis or trauma
*life threatening

42
Q

What is chronic DIC

A

Continuous or intermittent small triggers
-malignancy

Body can compensate
-liver clearance of FDPs
-increased production

43
Q

Where are platelets sequestered

A

Spleen

44
Q

What results will you see in labs with DIC

A

Thrombocytopenia
decreased fibrinogen
increased D-dimer

45
Q

What is the treatment of DIC

A

Treat underlying condition
Hemodynamic stabilization
Whole blood transfusion

46
Q

What is TTP

A

Thrombotic thrombocytopenia pupura

47
Q

What is thrombotic thrombocytopenia purpura

A

Thrombotic microangiopathy

48
Q

What are the risk factors for TTP

A

African ancestory
Obesity
Certain HLA subtypes

49
Q

What other things are associated with TTP

A

Renal, GI, CNS involvement
URI/ flu-like illness

50
Q

What will be seen on CBC with TTP

A

Severe hemolytic anemia and thrombocytopenia in otherwise healthy person

51
Q

What is the plasmic score in someone with TTP

A

6+

52
Q

What enzyme should be checked with TTP

A

ADAMTS13

53
Q

What is the treatment for hereditary TTP

A

Plasma infusion

54
Q

What is the treatment with acquired TTP

A

Urgent initiation of plasm exchange
(plasmapheresis)

55
Q

If someone has refractory TTP, how do you treat them

A

immunosuppression with steroids

56
Q

How many plasma exchanges typically need to happen to normalize platelet count in someone with TTP

A

11

57
Q

What is HUS

A

hemolytic-uremic syndrome

58
Q

What is the difference between HUS and TTP

A

HUS typically happens in children

59
Q

What is the prodrome for HUS

A

bloody diarrhea

60
Q

What typically causes HUS

A

Shiga-toxin (E.coli)
antibiotics
antidiarrheals

61
Q

What will be seen one workup with HUS

A

Hemolytic anemia
Thrombocytopenia
Renal injury

62
Q

What is the treatment for HUS

A

Supportive care
*Dialysis if severe renal impairment

63
Q

What is ITP

A

Idiopathic thrombocytopenia purpura

-Low platelet count in older adults (>6months)

64
Q

What gender is more effected by ITP

A

Females

65
Q

What is the difference between primary and secondary ITP

A

Primary: no identifiable trigger

Secondary: CA or infection (less likely)

66
Q

How will someone with ITP typically present

A

Asymptomatic
Fatigue
Petechiae / purpura
Epistaxis
hematomas

67
Q

How do you dx ITP

A

Diagnosis of exclusion

68
Q

What 2 diseases should be considered with someone who has ITP

A

HCV
HIV

69
Q

If someone with ITP is bleeding extensively, what treatment can be used

A

High dose steroids
IVIG
Platelet transfusion

70
Q

what causes HIT

A

adverse reaction to heparin

71
Q

What is type 1 HIT

A

non-immune related
-can continue heparin
*more common HIT

72
Q

What is type 2 HIT

A

Immune mediated
antibody production (happens day 5-14)
**More severe HIT

73
Q

Which form of heparin will more commonly cause HIT

A

UFH»>LMWH

74
Q

Why is UFH more likely to cause HIT

A

Bigger molecule
more antigenic

75
Q

What happens to platelets in someone with HIT

A

Unexplained drop in platelet count in patient on heparin

Steady drop, no fluctuation

Stable hgb/hct

76
Q

What are the 4 Ts for a HIT score

A

Thrombocytopenia
Timing of platelet count fall
Thrombosis or other sequela
OTher causes of thrombocytopenia

77
Q

How do you treat HIT

A

Discontinue heparin
Start alternate anticoag
Evaluate for HIT with PF4
–> If + confirm with serotonin assay

78
Q

What drug is a contraindication in someone with HIT

A

Coumadin
*precipitate skin necrosis and gangrene

79
Q

What is thrombocytosis

A

Elevated number of platelets

-megakaryocyte disorder

80
Q

What gene mutation is common with thrombocytosis

A

JAK2

81
Q

What is often associated with thrombocytosis

A

Malignancy

82
Q

How will thrombocytosis present

A

Often asymptomatic
Thromboses in weird sites
Abnormal bleeding
Splenomegaly

83
Q

What is erythromelalgia and what is it associated with

A

Painful burning of the hands
-can present with thrombocytosis

84
Q

What platelet count is indicative of thrombocytosis

A

> 400,000

85
Q

What is the treatment for thrombocytosis

A

Platelet count <500,000 is goal

1st line: Oral hydroxyurea

plateletpheresis if emergent platelet lowering is required

Clin Med hematology (6 decks)

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