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Hematology 2 > Platelet Qualitative Disorder > Flashcards

Platelet Qualitative Disorder Flashcards

1
Q

an instrument designed to measure
platelet function in a suspension of Citrated WB or PRP.

A

Aggregometer

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2
Q

TYPES OF PLATELET AGGREGOMETRY STUDIES

A
  1. Optical platelet aggregometry
  2. Whole blood platelet aggregometry
  3. Platelet lumi aggregometry
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3
Q

Sample used in Optical platelet aggregometry

A

PRP

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4
Q

Testing principle of Optical platelet aggregometry

A

Light transmittance

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5
Q

Baseline 0% Transmission scales down to 100% transmittance.

A

Optical platelet aggregometry

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6
Q

4 PHASES of Optical platelet aggregometry

A
  1. Baseline
  2. Primary-wave aggregation
  3. ADP/ATP release
  4. Second-wave aggregation
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7
Q

Sample used in Whole blood platelet Aggregometry

A

Whole blood (WB)

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8
Q

Testing principle of Whole blood platelet Aggregometry

A

Electrical impedance

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9
Q

As platelet aggregate, platelets collects on
the electrodes impeding the current. The change is amplified and recorded.

A

Whole blood platelet Aggregometry

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10
Q

Sample used in Platelet lumi Aggregometry

A

Whole blood (WB) or PRP

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11
Q

Testing principle of Platelet lumi Aggregometry

A

Chemiluminescence

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12
Q

Principle: Luciferin-luciferase enzyme (Reagent) added is oxidized by ATP which generates proportional chemiluminescence

A

Platelet lumi Aggregometry

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13
Q

Particularly sensitive to ATP release

A

Platelet lumi Aggregometry

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14
Q

AGONIST USED IN AGGREGOMETRY with Biphasic curves

A
  • Thrombin
  • ADP
  • Epinephrine
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15
Q

AGONIST USED IN AGGREGOMETRY with Monophasic curves

A
  • Collagen
  • Arachidonic Acid
  • Ristocetin
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16
Q

Receptors of Thrombin

A

PAR-1,PAR-4;
GP Iba & GP VI

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17
Q

Relatively unaffected by membrane disorders or enzyme deficiencies.

A

Thrombin

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18
Q

Receptors for ADP

A

P2Y1 & P2Y12

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19
Q

Most commonly used agonists.

A

ADP

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20
Q

Receptor for EPINEPHRINE

A

A2- Adrenergic Receptor

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21
Q

Cannot induce aggregation in storage pool disorders

A

EPINEPHRINE

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22
Q

Receptors for COLLAGEN

A

GP Ia/IIa & GPVI

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23
Q

Loss of response may indicate membrane abnormality, secretion defect or antiplatelet
drugs

A

COLLAGEN

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24
Q

Receptors for ARACHIDONIC ACID

A

TPa & TPb

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25
Q

Assess platelet eicosanoid pathway

A

ARACHIDONIC ACID

26
Q

Receptor for RISTOCETIN

A

GP Ib/IX/ V with vWf

27
Q

For the diagnosis of BSS and vWf

A

RISTOCETIN

28
Q

Disorders of Platelet Adhesion:

A

Bernard-Soulier Syndrome (Giant platelet syndrome)

29
Q

Inherited as Autosomal Recessive disorder in which the GP Ib/1X/V complex is missing from the platelet surface or exhibits abnormal function.

A

Bernard-Soulier Syndrome (Giant platelet syndrome)

30
Q

Inability to bind to VWF accounts for the inability of platelets to adhere to exposed sub endothelium and results to the bleeding characteristics of this disorder.

A

Bernard-Soulier Syndrome (Giant platelet syndrome)

31
Q

Laboratory features : Giant platelets

A

Bernard-Soulier Syndrome (Giant platelet syndrome)

32
Q

PLATELET AGGREGATION in

PLASMA OR PLATELET GRANULES

A
  • Ionized Calcium
  • Fibrinogen
33
Q

PLATELET AGGREGATION in

PLATELETS

A
  • GP IIb/IIIa
  • ATP/ADP
34
Q

Disorders of Platelet Aggregation:

A

Glanzmann Thrombasthenia

35
Q

Deficiency or abnormality of the platelet
membrane glycoprotein GPIlb/Illa complex.

A

Glanzmann Thrombasthenia

36
Q

Laboratory features : Platelet aggregometry.

Macrothrombocytopenia and prolonged bleeding time.

A

Bernard-Soulier Syndrome (Giant platelet syndrome)

37
Q

Normal platelet count and morphology.

Abnormal aggregation. Prolonged bleeding time.

A

Glanzmann Thrombasthenia

38
Q

Clinical Manifestations: Bleeding of all types

A

Glanzmann Thrombasthenia

39
Q

Dense Granule Deficiency:

A
  1. Hermansky-Pudlak syndrome
  2. Chediak- Higashi syndrome
  3. Wiskott-ALdrich syndrome
  4. Thrombocytopenia-absent
  5. radius (TAR) syndrome
40
Q

Alpha Granule Deficiency:

A
  1. Gray Platelet Syndrome
41
Q

Manifestations:
- Tyrosinase positive oculocutaneous albinism
- Lysosomal dysfunctions
- Ceroid-like depositions in the RES
- Profound platelet dense granule deficiency.
- Severe bleeding is RARE.

A

Hermansky- Pudlak Syndrome

42
Q

Hallmark: Swiss-cheese platelets. This abnormality consists of marked dilation and tortuosity of the surface-connecting tubular system

A

Hermansky- Pudlak Syndrome

43
Q

Manifestations:
- Partial oculocutaneous albinism
- Frequent pyogenic bacterial infections (severe immunology defects).
- Giant lysosomal granules
- Platelet dense granule deficiency (which leads to hemorrhage ).
- Lymphocytic proliferation in the liver, spleen and marrow.
- Macrophage accumulation in tissues.
- Severe pancytopenia
- Usually results to death in early age.

A

Chediak-Higashi Syndrome

44
Q
  • X-linked disease
  • Affects primarily males.
  • Mutations in the WAS gene on the short arm of the X chromosome. (Xp11.23)
A

Wiskott-Aldrich syndrome

45
Q

The WASp gene plays a crucial role in

A

actin cytoskeleton remodeling

46
Q

Classic form of WAS:

A

Eczema-thrombocytopenia immunodeficiency syndrome

47
Q
  • Immune dysfunction (susceptibility to bacterial, viral and fungal infections).
  • Microthrombocytopenia
  • Severe Eczema.
  • Bleeding episodes (mod-severe).
A

Eczema-thrombocytopenia immunodeficiency syndrome

48
Q
  1. Decreased platelet dense granules.
  2. Platelets are small- Microthrombocytes.
    Other instances where small platelets are hallmark features: TORCH Infections. ( bacterial infection that transmitted vertically from mother to the baby)
A

Wiskott-Aldrich syndrome

49
Q
  • Rare autosomal recessive disorder
  • Congenital absence of radial bones
  • Cardiac and skeletal abnormalities
  • Thrombocytopenia
  • Structural defects of platelet dense granules.
A

Thrombocytopenia with absent radii syndrome (TAR)

50
Q

mutation in the NBEAL2 gene (614169) on
chromosome 3p21.

A

Gray Platelet Syndrome

51
Q

Characterized by the specific absence of
morphologically recognizable alpha granules.

A

Gray Platelet Syndrome

52
Q
  • Lifelong mild bleeding tendencies.
  • Moderate thrombocytopenia
  • Fibrosis of the marrow
  • Large platelets with gray appearance on a wright-stained smear.
A

Gray Platelet Syndrome

53
Q

Membranes & precursors of gray platelet syndrome have

A

P-selectin and GPIIB/IlIA expressions

54
Q

a rare disorder in which both alpha and dense granules are deficient. Inherited in an autosomal dominant manner.

A

Alpha-Dense storage pool deficiency

55
Q

autosomal dominant bleeding disorder, this results to a deficiency in multimerin. Many alpha granules are degraded by proteases.

A

Quebec Platelet disorder

56
Q

DRUGS INFLUENCING PLATELET FUNCTIONS

A
  • Aspirin
  • Clopidogrel
  • Tirofiban
  • Atopaxar
57
Q

Inhibits platelet aggregation and secretion in
response to ADP, Epinephrine and collagen.

A

ASPIRIN

58
Q

inhibits prostaglandin synthesis by irreversible acetylation and inactivation of cyclooxygenase (COX-1)thereby inhibiting endoperoxide and thromboxane A2 which are necessary for platelet secretion.

A

Aspirin

59
Q

a thienopyridine derivative, binds specifically and irreversibly to the platelet P2RY12 purinergic receptor, inhibiting ADP-mediated platelet activation and aggregation.

A

CLOPIDOGREL

60
Q

a reversible, competitive inhibitor of GP Ilb/Illa receptors, exerting its effects via the prevention of the binding of fibrinogen and other ligands, resulting in the inhibition of platelet aggregation.

A

TIROFIBAN

61
Q

is a potent protease-activated receptor (PAR-1) antagonist targeting the G-coupled receptor and modulating thrombin-platelet-endothelial interactions.

A

Atopaxar (E5555)

Hematology 2 (18 decks)

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