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MTAP hematology Part 2 (Platelets > 3.1 Qualitative Platelet Disorders > Flashcards

3.1 Qualitative Platelet Disorders Flashcards

1
Q

Autosomal recessive disorder

a. Glanzmann thrombasthenia
b. Bernard-Soulier syndrome

A

a b

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2
Q

Deficiency or abnormality of the platelet membrane GP IIb/IIIa

a. Glanzmann thrombasthenia
b. Bernard-Soulier syndrome

A

a

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3
Q

Clinical manifestation: Petechiae, purpura, menorrhagia, gastrointestinal bleeding, hematuria

a. Glanzmann thrombasthenia
b. Bernard-Soulier syndrome

A

a

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4
Q

Deficient in Gp IB/IX/V

a. Glanzmann thrombasthenia
b. Bernard-Soulier syndrome

A

b

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5
Q

Lab features::

  • Thrombocytopenia
  • Giant Platelets (5-8um)

a. Glanzmann thrombasthenia
b. Bernard-Soulier syndrome

A

b

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6
Q

Lab features::

  • Normal platelet count, and morphology
  • abnormal clot retraction

a. Glanzmann thrombasthenia
b. Bernard-Soulier syndrome

A

A

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7
Q

Treatment of bleeding episodes requires the transfusion of normal platelet

a. Glanzmann thrombasthenia
b. Bernard-Soulier syndrome

A

a

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8
Q

Lab features::

Platelet aggregation test:
- Normal aggregation to ristocetin
- Defective agg to ADP, collagen, thrombin, epinephrine

a. Glanzmann thrombasthenia
b. Bernard-Soulier syndrome

A

a

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9
Q

Lab features::

Platelet aggregation test:
- Normal agg to ADP, epinephrine, collagen, arachidonic acid
- Defective agg to ristocetin
- Diminished agg to thrombin

a. Glanzmann thrombasthenia
b. Bernard-Soulier syndrome

A

b

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10
Q

Platelet transfusion are the therapy of choice (Plateletpheresis)

a. Glanzmann thrombasthenia
b. Bernard-Soulier syndrome

A

b

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11
Q

Storage pool disease is a problem in granules

T or F

A

T

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12
Q

Which of the following does not belong to dense granule deficiencies:

  • Hermansky-pudlak syndrome
  • Gray platelet syndrome
  • Chediak higashi syndrome
  • Wiskott-Aldrich syndrome
  • Thrombocytopenia-absent radius (TAR)
A

Gray platelet syndrome

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13
Q

Autosomal recessive disorder

a. Hermansky-pudlak syndrome
b. Chediak higashi syndrome
c. Wiskott-Aldrich syndrome
d. Thrombocytopenia-absent radius (TAR)

A

abd

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14
Q

Characterized by tyrosinase-positive oculocutaneous albinism, defective lysosomal function in a variety of ell types, ceroid-ike deposition in the cells of the R.E system and platelet dense granule deficiency

a. Hermansky-pudlak syndrome
b. Chediak higashi syndrome
c. Wiskott-Aldrich syndrome
d. Thrombocytopenia-absent radius (TAR)

A

a

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15
Q

Characterized by congenital absense oof the radial bones, numerous cardiac and other skeletal abnormalities and thrombocytopenia

a. Hermansky-pudlak syndrome
b. Chediak higashi syndrome
c. Wiskott-Aldrich syndrome
d. Thrombocytopenia-absent radius (TAR)

A

d

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16
Q

Characterized by partial oculocutaneous albinism, frequents pyogenic bacterial infections giant lysosomal granules in hematologic cells, platelet dense granule deficiency and hemorrhage

a. Hermansky-pudlak syndrome
b. Chediak higashi syndrome
c. Wiskott-Aldrich syndrome
d. Thrombocytopenia-absent radius (TAR)

A

b

17
Q

This disorder is accompanied by severe immunologic defects and progressive neurologic dysfunction

a. Hermansky-pudlak syndrome
b. Chediak higashi syndrome
c. Wiskott-Aldrich syndrome
d. Thrombocytopenia-absent radius (TAR)

A

b

18
Q

X- linked inheritance

a. Hermansky-pudlak syndrome
b. Chediak higashi syndrome
c. Wiskott-Aldrich syndrome
d. Thrombocytopenia-absent radius (TAR)

A

c

19
Q

T-cell function is defective due to abnormal cytoskeletal reorganization and platelets are structurally abnormal

a. Hermansky-pudlak syndrome
b. Chediak higashi syndrome
c. Wiskott-Aldrich syndrome
d. Thrombocytopenia-absent radius (TAR)

A

c

20
Q

Wiskott Aldrich syndrome:

The Classic form of WAS is characterized by a Triad:

I_____________ __________
E______
T_____________ with _______ ________

A

Immune deficiency
Eczema
Thrombocytopenia with small platelets

21
Q

An autosomal recessive disorder that has athe absence of morphologiallu recognizable alpha granules in platelets

A

Gray platelet syndome

22
Q

Gray platelet syndrome has large platelets that appears Gray on a What stain?

A

Wright Stain

23
Q

In Aquired plalelet dysfunction, Drugs are most common cause of acquired platelet dysfunction, what is the most frequent drug?

A

Aspirin

24
Q

A single 200mg dose of aspirin can irreversible acetylated 90% of the platelet ________________ (Inactive)

A

Cyclooxygenase

25
Q

Disorders that affect platelet function includes:

  • Myeloproliferatie neoplasms
  • Multiple myeloma
  • Waldenstrom macroglobulinemia
  • Cardiopulmonary bypass surgery
  • Liver disease
  • Uremia
  • Hereditary afirbrinogenemia

T or F

A

T

26
Q

Cyclooxygenase is needed for production of ________________ __

A

Thromboxane A2

27
Q

Hyperaagregable platelets includes:
- Hyperlipedemia,
- DM
- Peripheral arterial occlusive disease
- Acute arterial occlusion
- Myocardial infarction
- Stroke

T or F

A

T

28
Q

A hyperaggragable platelets that is an autosomal DOMINANT disorder that is characterized in response to ADP, epinephrine or both

Thromboembolic events are often associated with emotional stress

A

Sticky platelet syndrome

MTAP hematology Part 2 (Platelets (20 decks)

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