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OS 216 - Hematology Exam > Congenital Bleeding Disorders (Ma. Ysabel Lesaca-Medina, MD) > Flashcards

Congenital Bleeding Disorders (Ma. Ysabel Lesaca-Medina, MD) Flashcards

1
Q

Phases of hemostasis

A 
Vascular phases
Platelet phase
Coagulation phase
Clot retraction
Clot destruction
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2
Q

Automatic response of vessel to contain bleeding to site of injury

A

Transient vasoconstriction (reflex neurogenic and endothelin secretion)

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3
Q

Three As of the platelet phase

A

Adhesion
Activation
Aggregation

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4
Q

Platelet activators

A

ADP

Epinephrine

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5
Q

Serves as bridge in the aggregation of platelets

A

Fibrinogen

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6
Q

Goal of coagulation phase of hemostasis

A

Fibrin thombus formation (for stability)

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7
Q

Factors that play a role in clot destruction

A 
Thrombin
Factor XII (Hageman)
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8
Q

How is the intrinsic pathway activated?

A

Release of kininogens/kallikrein from injured surface

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9
Q

Pathway measured by PTT

A

Intrinsic pathway

Brad P(I)TT has a P(E)T!

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10
Q

What does automated PTT measure?

A

Factor XII

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11
Q

Trace the intrinsic pathway

A

XII -> XI -> IX + VIIIa -> X -> Common Pathway

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12
Q

Durations of intrinsic and extrinsic pathways

A

Intrinsic pathway: 20 – 30 seconds

Extrinsic pathway: 10 – 12 seconds

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13
Q

How is the extrinsic pathway activated?

A

Trauma

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14
Q

Trace the extrinsic pathway

A

Factor VII + Tissue Factor -> X -> Common pathway

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15
Q

Pathway measured by PT

A

Extrinsic pathway

Brad P(I)TT has a P(E)T!

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16
Q

Describe: Prothrombin Time

A

Time for III and CaCl to form a clot

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17
Q

Trace the common pathway

A

X -> Prothrombin to Thrombin -> Fibrinogen to Fibrin + Factor XIIIa -> Fibrin clot

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18
Q

Pathway measured by TT

A

Common pathway

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19
Q

How does deficiency in Factor XIII manifest

A

Normal hemostasis but persistent bleeding

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20
Q

Used to measure Factor XIII

A

Urea Clot Lysis Assay

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21
Q

Vitamin K-dependent factors

A

IX, X, VII and II (1972)

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22
Q

T/F: Factor VI has the shortest half-life at 8 hours

A

False

Factor VII. Factor VI does not exist.

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23
Q

First factor to get depleted

A

Factor VII

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24
Q

T/F: In acute Vit. K deficiency, only Factor VII decreases

A

True

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25
Q

T/F: Mucosal bleeding points to a coagulation problem.

A

False

It points to a platelet problem.

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26
Q

T/F: Deep tissue bleeding points to a coagulation problem.

A

True

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27
Q

When is there delayed onset bleeding?

A

Factor XIII deficiency

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28
Q

Liver disease affects the production of all clotting factors except these.

A

Factor VIII

vWF

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29
Q

T/F: A person with liver disease is more prone to thrombosis and exhibits a longer PT.

A

True

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30
Q

Given to the patient if vitamin K does not correct the PT.

A

Fresh frozen plasma

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31
Q

Examples of antiplatelet drugs

A

Aspirin

NSAIDs

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32
Q

Examples of anticoagulants

A

Heparin
Coumadin
Warfarin (combats vit. K)

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33
Q

Examples of antibiotics that can disrupt vit. K synthesis

A

Penicillin
Cephalosporin
Amphotericin

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34
Q

Useful in ruling in congenital bleeding disorders

A

Consanguinity

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35
Q

Criteria for menorrhagia

A

> 3 pads/day
Flooding
Hb < 10g/L

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36
Q

Common manifestation of hemophilia

A

Hemarthrosis

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37
Q

Indicative of a coagulation problem

A

Hemarthrosis

Intramuscular bleeds

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38
Q

Differentiate petechiae, purpura and ecchymoses on the basis of size

A

Petechiae - < 2 mm
Purpura - 2 mm to 1 cm
Ecchymoses - > 1 cm

39
Q

Used to check the status of fibrinogen

A

Thrombin time

40
Q

Blood is passed into a machine and the time it takes to measure hole closure corresponds to “bleeding time”

A

Platelet Function Analyzer (PFA)

41
Q

Used to determine whether a patient has factor deficiency or antibodies against clotting factors

A

Mixing studies

42
Q

T/F: If mixing patient blood with normal blood solves the problem, patient has antibody inhibitors in the blood.

A

False

Patient has factor deficiency.

43
Q

Finding if clot lyses quickly in urea clot lysis assay

A

Factor XIII deficiency

44
Q

Differentials if PT is elevated while PTT and platelet levels are normal

A

Early liver disease
Early Vit. K deficiency
Factor VII deficiency

45
Q

Differentials if PTT is elevated while PT and platelet levels are normal

A

Factor VIII deficiency (hemophilia or vWD)
Factor IX, XI or XII deficiency
Inhibitors

46
Q

Differentials if both PTT and PT are elevated and platelet levels are normal

A

Late liver disease
Late Vit. K deficiency
Massive transfusion

47
Q

Differentials if PTT and TT are elevated and platelet and PT levels are normal

A

Heparin

48
Q

Differentials if PTT, TT, PT and platelet levels are all normal

A 
vWD
Platelet function disorder
Mild factor deficiency (VIII, IX, XI or XIII)
Collagen disorder
Vit. C deficiency
49
Q

Differentials if platelet levels are decreased

A

CAMT, TAR, BSS, WAS, GPOS or ITP

Infection

50
Q

Most common inherited bleeding disorder

A

Von Willebrand’s disease

51
Q

Rare bleeding disorder where there is no megakaryocyte in the bone marrow

A

Congenital Amegakaryocytic Thrombocytopenia (CAMT)

52
Q

Rare bleeding disorder where alpha-granules are lacking and patient is prone to infection

A

Gray Platelet Syndrome (GPS)

53
Q

Rare bleeding disorder involving multiple congenital anomalies and thrombocytopenia

A

Thrombocytopenia with Absent Radius (TAR)

54
Q

Rare bleeding disorder where platelets are small

A

Wiskott Aldrich Syndrome (WAS)

55
Q

Most common severe congenital bleeding disorder

A

Hemophilia

56
Q

Severity classifications for hemophilia

A

Severe - <1% activity
Moderate - 1 - 5% activity
Mild - 5 - 50% activity

57
Q

Types of hemophilia

A

Hemophilia A - FVIII

Hemophilia B - FIX

58
Q

Characteristics of hemophilia carriers

A

Factor VIII usually below normal levels
Mild bruising
Heavy menstrual periods

59
Q

Complications of hemophilia

A

Hemarthroses
Intracranial hemorrhage
Intramuscular hematomas

60
Q

Common sites of intramuscular hematomas

A

Anterior forearm

Anterior tibial compartments

61
Q

General aim of hemophilia management

A

Correct FVIII to normal limits

62
Q

Synthetic vasopressin analog that causes transient rise in FVIII and vWF and used for mild cases of hemophilia

A

Desmopressin or DDAVP

63
Q

Used to treat moderate to severe hemophilia B

A

Fresh frozen plasma or cryosupernate (if available)

64
Q

Used to treat moderate to severe hemophilia A in the absence of FVIII

A

Cryoprecipitate

65
Q

Not used in treatment of hemophilic patients < 6 months old due to increased risk of inhibitor development

A

Preventive Factor VIII infusions

66
Q

Quantifies inhibitor formation

A

Bethesda units (BU)

67
Q

Define: 1 Bethesda unit (1 BU)

A

Amount of antibodyt hat neutralizes 50% of FVIII or IX present after 2 hours incubation at 37 degrees Celsius

68
Q

Treatment for inhibitor patients with BU < 5

A

Increase dose of FVIII

69
Q

Treatment for inhibitor patients with BU > 5

A

Bypass agents
Prothrombin complex concentrates
Immune tolerance induction
Immunosuppressive agents (rituximab)

70
Q

Methods of immune tolerance induction (ITI)

A

Malmo Regimen
Van Cremeld Regimen
Bonn Regimen

71
Q

Infectious diseases hemophilic patients are still at risk for during treatment

A

Hepatitis A
Creutzfeld-Jakob Disease
Parvovirus B19

72
Q

Complication of gene transfer treatment of hemophilia

A

Hepatitis with concomitant destruction of transferred gene

73
Q

Types of Von Willebrand’s Disease (vWD)

A

Type 1 - Classic partial quantitative deficiency
Type 2 - Qualitative deficiency
Type 3 - Near complete deficiency

74
Q

Mode of inheritance of Von Willebrand’s Disease (vWD)

A

Autosomal dominant (mostly), but can be autosomal recessive

75
Q

Most common cause of acquired vWD

A

Presence of antibody to vWF

76
Q

T/F: Theoretically, there should be equal cases of vWD in both sexes

A

True

77
Q

T/F: Sometimes, PTT is elevated in Type 2 vWD

A

False

Type 3 vWD

78
Q

T/F: Platelet count may be decreased in vWD Types 2 and 3.

A

True

79
Q

T/F: Blood type O has lower levels of vWF

A

True

80
Q

Used for definitive testing of vWD

A

vWF assay

81
Q

Treatment of vWD

A

Desmopressin (types 1 & 2)

82
Q

Mode of inheritance of rare congenital coagulation factor deficiencies

A

Autosomal recessive

83
Q

Congenital coagulation factor deficiency that causes the most bleeding

A

Factor X deficiency

Factor XIII deficiency

84
Q

SSx of rare coagulation disorders

A 
Umbilical stump bleeding
Delayed cord separation
Intracranial or intestinal hemorrhage
Muscle hematomas
Easy bruising
Prolonged bleeding following prick
85
Q

Disorder characterized by absence of GPIb/IX receptor

A

Bernard-Soulier Syndrome (BSS)

86
Q

Mode of inheritance of Wiskott-Aldrich Syndrome

A

X-linked

87
Q

Mode of inheritance of Gray Platelet Syndrome

A

Autosomal dominant or recessive

88
Q

Mode of inheritance of Bernard-Soulier Syndrome

A

Autosomal recessive

89
Q

Disorder characterized by defective GP IIb/IIIa interaction

A

Glanzmann Thrombasthenia (GT)

90
Q

How much Factor VIII should be infused in non-life threatening bleeds?

A

25 - 40 U/kg

91
Q

How much Factor VIII should be infused in life-threatening bleeds?

A

50 U/kg

92
Q

T/F: vW antigen levels are normal in Type II vWD

A

True

93
Q

Characteristics of Factor VII responsible for isolated prolonged PT in early liver disease or Vitamin K deficiency

A

Short half-life

OS 216 - Hematology Exam (8 decks)

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