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MTAP 2 - HEMA (Readings) > Chap 37: Qualitative Disorder of Platelets and Vasculature > Flashcards

Chap 37: Qualitative Disorder of Platelets and Vasculature Flashcards

1
Q

Excessive bruising and superficial (mucocutaneous) bleeding in a patient whose platelet count is normal suggest an

A

Acquired or congenital disorder of platelet function

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2
Q

Disorders of Platelet Aggregation

A
  • Glanzmann thrombasthenia
  • Hereditary afibrinogenemia
  • Acquired defects of platelet aggregation (Acquired von Willebrand disease, Acquired uremia)
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3
Q

Disorders of Platelet Adhesion

A
  • Bernard-Soulier syndrome
  • Von Willebrand disease
  • Acquired defects of platelet adhesion:
    (Myeloproliferative and lymphoproliferative disorders, dysproteinemias
    Antiplatelet antibodies
    Cardiopulmonary bypass surgery
    Chronic liver disease
    Drug-induced membrane modification)
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4
Q

Disorder of Platelet Secretion

A
  • Storage pool diseases
  • Thromboxane pathway disorders
  • Hereditary aspirin-like defects (Cyclooxygenase or thromboxane synthetase deficiency)
  • Drug inhibition of the prostaglandin pathways
  • Drug inhibition of platelet phosphodiesterase activity
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5
Q

Described as bleeding disorder associated with abnormal in vitro clot retraction and a normal platelet count

A

Glanzmann Thrombasthenia

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6
Q

Is the process by which the volume of a formed clot is reduced through contraction of the intracellular actin-myosin cytoskeleton of activated platelets incorporated in the clot.

A

Clot retraction

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7
Q

GT is inherited and most frequently seen in population with a high degree of ?

A

Consanguinity

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8
Q

Hemorrhagic manifestation og GT includes:

A

petechiae, purpura, menorrhagia, gastrointestinal bleeding, and hematuria

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9
Q

Biochemical lesion responsible for the disorder is a deficiency or abnormality of what platelet membrane glycoprotein

A

(GP) IIb/IIIa (integrin aIIb3) complex,

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10
Q

A membrane receptor capable of binding fibrinogen, von Willebrand factor (VWF), fibronectin, and other adhesive ligand

A

GP IIb/IIIa

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11
Q

T/F: Patients with type 2 GT disease are less affected by abnormal clot retraction and fibrinogen binding.

A

TRUE

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12
Q

Genetic mutations of GT are distributed widely over what genes?

A

ITGA2b or ITGb3

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13
Q

What chromosome codes for GP IIb/IIIa

A

Chromosome 17

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14
Q

Lab feature for GT

A

Normal Platelet count
Normal platelet morphology
Lack platelet aggregation
Normal ristocetin-induced binding of VWF

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15
Q

One of the few forms of platelet dysfunction in which hemorrhage is severe and disabling.

A

Thrombasthenia

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16
Q

Patients with GT should avoid

A

Anticoagulants and antiplatelet agents such as aspirin and nonsteroidal anti-inflammatory drugs

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17
Q

Treatment to control or prevent bleeding caused by GT

A
  • Hormonal therapy (norethindrone acetate)
  • Antifibrinoytic therapy
  • Recombinant Factor VIIa
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18
Q

A rare disorder of platelet adhesion that usually manifests in infancy or childhood with hemorrhage characteristic of defective platelet function: ecchymoses, epistaxis, and gingival bleeding

A

Bernard-Soulier (Giant Platelet) Syndrome

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19
Q

BSs in inherited as an autosomal recessive in what glycoprotein complex?

A

GP Ib/IX/V

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20
Q

Essential to normal function because it contains binding sites for VWF and thrombin.

A

GP Iba

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21
Q

The most frequent forms of BSS involve defects in

A

GP Iba

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22
Q

The clotting activity of this factor is reduced in BSS

A

Factor VIII

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23
Q

Lab features of BSS

A
  • Normal aggregation responses to ADP, epinephrine, collagen and arachidonic acid
  • Do not respond to ristocetin
  • Diminished response to thrombin
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24
Q

Platelets used to treat BSS should be _ to reduce alloimmunization

A

Pre-storage leukoreduced

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25
Q

BSS is characterized by

A

Large platelets and thrombocytopenia

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26
Q

Platelet structure has abnormal microtubule distribution

A

May-Hegglin anomaly

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27
Q

Platelets are spherical and have a prominent surface-connected canalicular system

A

Epstein syndrome

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28
Q

Most common of the hereditary platelet function defects. Patients with these disorders present with mucocutaneous hemorrhage and hematuria, epistaxis, and easy and spontaneous bruising

A

Storage pool and release reaction defects

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29
Q

Dense granules are the storage site of? (4)

A
  • Serotonin
  • Nucleotide (ADP and ATP)
  • Calcium
  • Pyrophosphate
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30
Q

T/F: Dense granule deficiency does not typically result in a serious hemorrhagic problem. Bleeding is usually mild and most often is limited to easy bruising

A

TRUE

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31
Q

Substances that induce a primary wave of aggregation, but a secondary wave is missing

A

Epinephrine and Low-dose ADP

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32
Q

Examples of Dense Granule Deficiency

A
  • Hermansky-Pudlak syndrome
  • Chédiak-Higashi syndrome
  • Wiskott-Aldrich syndrome
  • Thrombocytopenia–absent radius (TAR) syndrome
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33
Q

Autosomal recessive disorder characterized by tyrosinase- positive oculocutaneous albinism, defective lysosomal function in a variety of cell types, ceroid-like deposition in the cells of the reticuloendothelial system, and a profound platelet dense granule deficiency

A

Hermansky-Pudlak syndrome

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34
Q

Mutations in at least _ genes individually can give rise to Hermansky-Pudlak Syndrome

A

Seven

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35
Q

Can be used to treat skin wounds that fail to spontaneously clot

A

Thrombin-soaked Gelfoam

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36
Q

The unique morphologic abnormality describe in patient with Hermansky-Pudlak syndrome

A

marked dilation and tortuosity of the surface-connecting tubular system (Swiss Cheese Platelet)

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37
Q

Characterized by partial oculocutaneous albinism, frequent pyogenic bacterial infections, giant lysosomal granules in cells of hematologic and nonhematologic origin, platelet dense granule deficiency, and hemorrhage.

A

Chédiak-Higashi syndrome

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38
Q

Gene for the Chédiak-Higashi syndrome protein is lo- cated on

A

Chromosome 1

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39
Q

This disorder progresses to an accelerated phase that is marked by lymphocytic proliferation in the liver, spleen, and marrow with macrophage accumulation in tissues

A

Chédiak-Higashi syndrome

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40
Q

Disease caused by mutations in the WAS gene on the short arm of the X chromosome Xp11.23 that encodes for a 502-amino acid protein

A

Wiskott-Aldrich syndrome (WAS)

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41
Q

Plays a crucial role in actin cytoskeleton remodeling

A

Wiskott-Aldrich syndrome protein (WASp)

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42
Q

classic form of WAS, characterized by susceptibility to infections associated with immune dysfunction, with recurrent bacterial, viral, and fungal infections, microthrombocytopenia, and severe eczema

A

Eczema-thrombocytopenia immunodeficiency syndrome

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43
Q

Individuals with WAS lack the ability to make anti- polysaccharide antibodies, which results in a propensity for

A

Pneumoccocal sepsis

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44
Q

A diagnostic feature of WAS platelets

A
  • Dense granules is decreased
  • Small platelets (microthrombocytes)
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45
Q

Other than in WAS, such small platelets are seen only in association with

A

TORCH (toxoplasma, other agents, rubella virus, cytomegalovirus, herpesvirus) infections

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46
Q

Other lab features of WAS

A
  • Decreased aggregation in response to ADP, collagen and epinephrine
  • Lack of 2ndary wave of aggregation
  • Normal thrombin
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47
Q

Rare autosomal recessive disorder characterized by the congenital absence of the radial bones (the most pronounced skeletal abnormality), numerous cardiac and other skeletal abnormalities, and thrombocytopenia

A

Thrombocytopenia with absent radii syndrome( TAR)

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48
Q

This cell may be decreased in number, immature, or normal in TAR

A

Marrow megakaryocytes

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49
Q

The storage site for proteins produced by the megakaryocyte (e.g., platelet-derived growth factor, thrombospondin, and platelet factor 4)

A

Alpha granules (a-granules)

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50
Q

How may a-granules are found per platelet? Hence these are responsible for the granular appearance of platelets on stained blood films

A

50-80 a-granules

51
Q

Rare disorder characterized by the specific absence of morphologically recognizable a-granules in platelets

A

Gray platelet syndrome

52
Q

Characterized by lifelong mild bleeding tendencies, moderate thrombocytopenia, fibrosis of the marrow, and large platelets whose gray appearance on a Wright-stained blood film

A

Gray platelet syndrome

53
Q

This has been used to control bleeding in patient with Gray platelet syndrome

A

Cryoprecipitate

54
Q

Shorten the bleeding
time test and has been used as successful prophylaxis during a dental extraction procedure.

A

Desmopressin acetate

55
Q

An autosomal dominant bleeding disorder that results from a deficiency of multimerin (a multimeric protein that is stored complexed with factor V in a-granules).

A

Quebec platelet disorder

56
Q

Converted to intermediate prostaglandins by cyclooxygenase and to thromboxane A2 by thromboxane synthase

A

Arachidonic acid

57
Q

Several acquired or congenital disorders of platelet secretion can be traced to structural and functional modifications of

A

Arachidonic acid pathway enzymes

58
Q

Required for storage granule secretion and maxi- mal platelet aggregation in response to epinephrine, ADP, and low concentrations of collagen.

A

Thromboxane A2

59
Q

Hereditary absence or abnormalities of the components of the thromboxane pathway are usually termed

A

Aspirin-Like defects

60
Q

One of the collagen receptors in the platelet membrane

A

A2B1 (GP Ia/IIa) integrin

61
Q

Are members of the seven-transmembrane domain (STD) family of G protein-linked receptors

A

P2Y1 and P2Y12

62
Q

The receptor that is linked to an ion channel that facilitates calcium ion influx.

A

P2X1

63
Q

Is thought to mediate calcium mobilization and shape change in response to ADP

A

P2Y1

64
Q

Asociated with decreased platelet activation and aggregation in response to epinephrine are known

A

Congenital defect of the a2-adrenergic (epinephrine) receptor

65
Q

Defects represent a heterogeneous group of disorders in which all elements of the thromboxane pathway are normal, but insufficient calcium is released from the dense tubular system

A

Calcium mobilization

66
Q

A very rare autosomal recessive disorder of calcium-induced membrane phospholipid scrambling (necessary for coagulation factor assembly) and thrombin generation on platelets.

A

Scott syndrome

67
Q

In Scott syndrome, platelet secrete and aggregate normally but do not transport

A

Phosphatidylserine and phosphatidylethanolamine

68
Q

Asymmetry of resting platelet is maintained by the enzyme

A

Aminophospholipid translocase

69
Q

Autosomal dominant disorder characterized by a mild bleeding tendency due to platelet dysfunction, thrombocytopenia, anemia, functional asplenia, and other constitutive disorders

A

Stormorken syndrome

70
Q

Platelets are always in an “activated” state and express phosphatidylserin

A

Stormorken Syndrome

71
Q

Most frequent cause of acquired platelet dysfunction is

A

Drug ingestion

72
Q

A single _ can irreversibly acetylate 90% of the platelet cyclooxygenase

A

200-mg dose of aspirin

73
Q

Endothelial cells also produce this another potent platelet inhibitor

A

Nitric Oxide (NO)

74
Q

T/F: Patients who do not respond well to aspirin have worse cardiovascular outcomes than patients who respond well

A

TRUE

75
Q

One system that provides measurement of a patient’s response to antiplatelet medication

A

VerifyNow (IL-Werfen)

76
Q

Group of drugs that decreases platelet function

A

ibuprofen, ketoprofen, fenoprofen, naproxen, and sulfinpyrazone.

77
Q

P2Y12 (ADP) receptor inhibitors

A

thienopyridine derivatives clopidogrel and prasugrel; their predecessor, ticlopidine; the nucleoside ticagrelor; and the nucleotide mimetic cangrelor

78
Q

A prodrug that requires conversion to the active drug by the 2C19 (a.k.a. CYP2C19) isoform of P450 in the liver.

A

Clopidogrel

79
Q

Third-generation thienopyridine derivative with the same mechanism of action as clopidogrel

A

Prasugrel

80
Q

Not a prodrug and therefore does not require bioactivation. Because it is rapidly absorbed

A

Ticagrelor

81
Q

a reversible, nucleotide mimetic P2Y12 inhibitor that, like ticagrelor, does not undergo metabolic activation

A

Cangrelor

82
Q

Approved for use as an adjunct treatment for reducing the risk of myocardial infarction, repeat coronary intervention, and stent thrombosis in patients undergoing per- cutaneous coronary intervention

A

Cangrelor

83
Q

inhibits thrombin-induced platelet aggregation by blocking the ligand-binding site on PAR-1.

A

Vorapaxar

84
Q

inhibitor of platelet phosphodiesterase, the enzyme responsible for converting cyclic adenosine monophosphate (cAMP) to AMP. Inhibition of phosphodiesterase allows the accumulation of cAMP in the cytoplasm; elevation of cytoplasmic cAMP is inhibitory to platelet function

A

Dipyridamole

85
Q

non-b-lactam antibiotic that may inhibit platelet aggregation when high concentrations are present in the blood.

A

Nitrofurantoin

86
Q

partially hydrolyzed, branched-chain polysaccharides of glucose

A

Dextrans

87
Q

Both drugs are commonly used as plasma ex- pandes

A

Dextran 70 and Dextran 40

88
Q

A synthetic glucose polymer with a mean molecular mass up to 450,000 Daltons that is used as a plasma expander.

A

Hydroxyethyl starch, or hetastarch

89
Q

Drugs used to regulate cardiovascular function that seem to be able to cause a decrease in platelet secretion and aggregation

A

Nitroglycerin, nitroprusside, propranolol, and isosorbide dinitrate

90
Q

Patient taking this may have decreased secretion and aggregation responses

A

Phenothiazine and tricyclic antidepressant

91
Q

Chronic myeloproliferative neoplasms (MPNs) include

A

polycythemia vera, chronic myelogenous leukemia (CML), essential thrombocythemia (ET), and myelofibrosis with myeloid metaplasia

92
Q

Patient with MPNs have been reported with having platelets that are

A

abnormal in shape, decreased procoagulant activity, decreased number of secretory granules and shortened survival

93
Q

Most common functional abnormalities are

A

Decreased aggregation and secretion in response to epinephrine, ADP, and collagen

94
Q

Platelet dysfunction results from coating of the platelet membrane by paraprotein and does not depend on the type of paraprotein present is seen on

A

IgA myeloma and Waldenstrom macroglobulinemia

95
Q

The use of _ during cardiac surgery induces thrombocytopenia and a severe platelet function defect

A

Cardiopulmonary bypass

96
Q

Reduction in clotting proteins, reduction of proteins in the natural anticoagulant pathways, dysfibrinogenemia, and excessive fibrinolysis are seen on patient with

A

Liver disease

97
Q

Commonly accompanied by bleeding caused by platelet dysfunction

A

Uremia

98
Q

What acid is present in uremia which is a result of inhibition of the urea cycle

A

guanidinosuccinic acid (GSA)

99
Q

An independent cause of prolonged bleeding time, and the severity of anemia in uremic patients correlates with the severity of renal failure

A

Anemia

100
Q

Not truly a platelet function disorder, but platelets do not exhibit normal function in the absence or near-absence of fibrinogen

A

Congenital afibrinogenemia

101
Q

Can be used to treat bleeding episodes in Congenital afibrinogenemia

A

Cryoprecipitate or Fibrinogen concentrate

102
Q

Autosomal dominant, thrombophilic disorder that is associated with venous and arterial thromboembolic events.

A

Sticky platelet syndrome

103
Q

Sticky platelet syndrome is characterized by

A

Hyperaggregable platelets in response to ADP, epinephrine, or both

104
Q

Is an indicator of abnormally increased platelet reactivity and often accompanies increased sensitivity to platelet agonists

A

Spontaneous aggregation (aggregation in response to in vitro stirring only)

105
Q

The esult of in vivo stimulation by thrombin and other agonists resulting in in vivo release of granule contents is called

A

Acquired storage pool disease (exhausted platelets)

106
Q

Vascular defect of this disorder is characterized by thin-walled blood vessels with a discontinuous endothelium, inadequate smooth muscle, and inadequate or missing elastin in the surrounding stroma

A

Hereditary Hemorrhagic Telangiectasia

107
Q

An almost universal finding, and symptoms almost always worsen with age (seen on Telangiectasia)

A

Epistaxis

108
Q

Common in older men and women

A

Cherry-red hemangiomas

109
Q

Louis bar syndrome

A

Ataxia-Telangiectasia

110
Q

Seen in association with chronic liver disease and pregnancy

A

Chronic Actinic Telangiectasia

111
Q

A giant cavernous hemangioma (vascular tumor), thrombocytopenia, and a bleeding diathesis.

A

Kasabach and Merritt

112
Q

May become engorged with blood and resemble hematomas.

A

External Hemangiomas

113
Q

May be transmitted as an autosomal dominant, recessive, or X-linked trait. May be manifested by hyperextensible skin, hypermobile joints, joint laxity, fragile tissue and bleeding tendency

A

Ehlers-Danlos Syndrome

114
Q

This disorder generally can be ascribed to defects in collagen production, structure, or cross- linking, with resulting inadequacy of the connective tissues

A

Ehlers-Danlos syndrome

115
Q

Applied to a group of nonthrombocytopenic purpuras characterized by apparently allergic manifestations, including skin rash and edema

A

Allergic purpura or anaphylactoid purpura

116
Q

Condition where acute IgA-mediated disorder with widespread generalized vasculitis involving the skin, joints, kidneys, gastrointestinal tract, and, less commonly, the lungs.

A

Henoch-Schönlein purpura

117
Q

Has been associated with cer- tain foods and drugs, cold, insect bites, and vaccination

A

Allergic purpura

118
Q

Henoch-Schönlein purpura is primarily a disease of children, occurring most commonly in children

A

3 to 7 years of age

119
Q

High concentrations of __ can cause severe hemorrhagic manifestations as a result of a combination of hyperviscosity and platelet dysfunction

A

Paraprotein

120
Q

A fibrous protein consisting of rigid, linear, non- branching, aggregated fibrils approximately 7.5 to 10 nm wide and of indefinite length

A

Amyloid

121
Q

The deposition of abnormal quantities of amyloid protein in tissues, may be primary or secondary, and localized or systemic

A

Amyloidosis

122
Q

Occurs more commonly in elderly men than in women and is due to a lack of collagen support for small blood vessels and loss of subcutaneous fat and elastic fibe

A

Senile Purpura

123
Q

Purpura associated with drug-induced vasculitis occurs in the presence of functionally adequate platelets

A

Drug-Induced Vascular Purpuras

MTAP 2 - HEMA (Readings) (2 decks)

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