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🩸Hema🎀 > 4: Aualitative Disorders > Flashcards

4: Aualitative Disorders Flashcards

1
Q

Plasma count in Platelet Rich Plasma

A

200-300 x 10^9/L

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2
Q

Sample used in Optical Platelet Aggregometry

A

PRP

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3
Q

Principle used in Optical Platelet Aggregometry

A

Light transmittance aggregometry

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4
Q

Baseline 0% transmission scaled down to 100% transmittance

A

Optical Platelet Aggregometry

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5
Q

5 phases of Optical Platelet Aggregometry

A
  1. Baseline
  2. Shape change
  3. Primary-wave aggregation
  4. ADP/ATP release
  5. Second-wave aggregation
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6
Q

Anticoagulant used in Whole Blood Aggregometry

A

Citrate

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7
Q

Sample used in Whole Blood Aggregometry

A

Whole blood

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8
Q

Principle used in Whole Blood Aggregometry

A

Electrical impedance

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9
Q

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

A

Whole Blood Aggregometry

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10
Q

Sample used in Platelet Lumi Aggregometry

A

WB and PRP

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11
Q

Principle used in Platelet Lumi Aggregometry

A

Chemiluminescence

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12
Q

What reagent is added in Platelet Lumi Aggregometry test that is oxidized by ATP which generates proportional chemiluminescence

A

Luciferin-luciferase enzyme

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13
Q

Typically the first agonist used in Platelet Lumi Aggregometry because it induces full secretion

A

Thrombin

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14
Q

Biphasic agonists

A

Thrombin
ADP
Epinephrine

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15
Q

Monophasic agonists

A

Collagen
Arachidonic acid
Ristocetin

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16
Q

What curve is related to the secretion of the granules

A

Biphasic curve

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17
Q

What curve only produces primary wave and is used to assess platelet aggregation

A

Monophasic curve

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18
Q

Receptors of thrombin

A

PAR1 & PAR4
GPIba & GPVI

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19
Q

Receptors of ADP

A

P2Y1 & P2Y12

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20
Q

Receptors of Epinephrine

A

A2-adrenergic

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21
Q

Agonist that is relatively unaffected by membrane disorders or enzyme deficiencies

A

Thrombin

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22
Q

Most commonly used agonist

A

ADP

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23
Q

Cannot induce aggregation in storage pool disorder or eicosanoid synthesis pathway defects no matter how high its concentration

A

Epinephrine

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24
Q

Does not work in WB aggregometry test for abnormalities of membrane binding sites and the eicosanoid synthesis pathway

A

Epinephrine

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25
Q

Receptors of Collagen

A

GPIa/GPIIa & GPVI

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26
Q

Receptos of Arachidonic acid

A

TPa and TPb

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27
Q

Receptors of Ristocetin

A

GPIb/IX/V with vWF

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28
Q

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

A

Collagen

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29
Q

Agonist that assess platelet eicosanoid synthesis pathway

A

Arachidonic acid

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30
Q

Agonist used for the diagnosis of BSS and vWD

A

Ristocetin

31
Q

A disease that cannot be corrected by additional of normal plasma or cryo

A

Bernard-Soulier Syndrome (Giant platelet syndrome)

32
Q

The most frequent forms of BSS involves defects in ______ synthesis or expression

33
Q

Variants of BSS:

A
  1. Normal but impaired functionality
  2. Mutation that cause poor binding
  3. Antibody to GPIb/V that case pseudo-BSS
34
Q

Laboratory feature of BSS:

A
  • Giant platelets
  • Macrothrombocytopenia (plts are large and fewer)
  • Prolonged bleeding time
35
Q

Treatment of choice of BSS

A

Platelet transfusion

note: must be leukoreduced to reduce alloimmunization

36
Q

What therapy should be avoided in BSS

A

Antiplatelet therapy

37
Q

Therapy in BSS that is useful for mucosal bleeding

A

Antifibrinolytic therapy

38
Q

Dominant type of BSS

A

BOLZANO TYPE

39
Q

In Glanzmann Thrombasthenia, GPIIb/IIIa complex is responsible for binding

A

fibrinogen
vWF
fibronectin
and other adhesive ligands

40
Q

0.5% of normal GPIIb/IIIa

A

Type 1 Glanzmann Thrombasthenia

41
Q

10-20% of normal GPIIb/IIIa

A

Type 2 Glanzmann Thrombastenia

42
Q

More severe type of Glanzmann Thrombastenia, with platelets lacking GPIIb/IIIa as well as intra platelet fibrinogen

43
Q

Highly recommend treatment for Glanzmann Thrombasthenia

A

Platelet transfusion

44
Q

What should be avoided in the treatment of Glanzmann Thrombasthenia

A

Anticoagulant and antiplatelet agents

45
Q

Treatment for Menorrhagia in Glanzmann thrombastenia

A

Hormonal therapy (Norethindrone acetate)

46
Q

Clot retraction of Glanzmann and BSS

A

Glanzmann: Abnormal
BSS: Normal

47
Q

Platelet glycoprotein defect of Glanzmann Thrombastenia

A

GPIIb/IIIa

48
Q

Platelet glycoprotein defect of BSS

49
Q

Chromosome 19 mutation

A

Hermansky-Pudlak Syndrome

50
Q

Manifestations:

  1. Tryosinase positive oculocutaneous albinism
  2. Lysosomal dysfunction
  3. Ceriod-like depositions in the RES
  4. Profound platelet dense granule deficiency
A

Hermansky-Pudlak Syndrome

51
Q

For extensive surgery and prolonged bleeding of Hermansky-Pudlak syndrome, administer

A

Packed red cells and platelets

52
Q

Hallmark of Hermansky-Pudlak Syndrome

A

Swiss-cheese platelets

53
Q

This abnormality consists of marked dilation and tortuosity of the surface-connecting tubular system

A

Swiss-cheese platelets

54
Q

Mutations in Chromosome 1

A

Chediak-Higashi Syndrome

55
Q

What gene is responsible for Chediak-Higashi Syndrome

A

CHY-LYST-1

56
Q

Manifestations:

  1. Partial oculocutaneous albinism
  2. Frequent pyogenic bacterial infections
  3. Giant lysosomal granules
  4. Platelet dense granule deficiency
A

Chediak-Higashi Syndrome

57
Q

Has severe pancytopenia

A

Chediak-Higashi Syndrome

58
Q

Macrophage accumulation in tissues

A

Chediak-Higashi Syndrome

59
Q

Usually results to death in early age

A

Chediak-Higashi Syndrome

60
Q

Mutations in the WAS gene on the short arm of the X chromosome (Xp11.23)

A

Wiskott-Aldrich Syndrome

61
Q

Classic form of WAS

A

Eczema-thrombocytopenia Immunodeficiency Syndrome

62
Q

Manifestations of Eczema-thrombocytopenia Immunodeficiency Syndrome

A
  • Immune dysfunction
  • Microthrombocytopenia
  • Severe Eczema
  • Bleeding episodes
63
Q

Hallmark of Wiskott-Aldrich Syndrome

A

TORCH infections

64
Q

The platelets are small - Microthrombocytes

A

Wiskott-Aldrich Syndrome

65
Q

Congenital absence of radial bones

A

Thrombocytopenia with Absent Radius Syndrome (TAR)

66
Q

How many alpha granules are present in platelets

67
Q

Characterized by large platelets

A

Gray Platelet Syndrome

68
Q

Mutations in the NBEAL2 gene (614169) on chromosome 3p21

A

Gray Platelet Syndrome

69
Q

Autosomal dominant bleeding disorder which results to a deficiency in multimerin

A

Quebec Platelet Disorder

70
Q

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

71
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

72
Q

Binds specifically and irreversibly to the platelet P2Y12 purigenic receptor inhibiting ADP-mediated platelet activation and aggregation

A

Clopidogrel

73
Q

Is reversible, competitive inhibitor of GP IIb/IIIa receptors, exerting its effects via the prevention of the binding of fibrinogen and other ligands, resulting in the inhibition of platelet aggregation

74
Q

A potent protease-activated receptor (PAR-1) antagonist targeting G-coupled receptor and modulating thrombin-platelet endothelial-interactions

A

Atopaxar (E5555) - Benzimidazole receptor (PAR1)

🩸Hema🎀 (20 decks)

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