Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Hematology 2 > Qualitative disorders > Flashcards

Qualitative disorders Flashcards

1
Q
  • Decreased/absence of gp IIb/IIIa (fibrinogen receptor)
  • Seen in consanguinity
  • Bruising, epistaxis, mucus membrane bleeding, menorrhagia, GIT
    bleeding, and hematuria

a. Glanzmann’s
b. Berard-Soulier
c. Quebec
d. Gray platelets

A

Glanzmann’s Thrombasthenia

Treatment is transfusion (single-donor)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q
  • Decreased/lacks aggregation with epinephrine, ADP, collagen, and thrombin
  • Normal aggregation response with ristocetin
  • Lack of clot retraction

a. Glanzmann’s
b. Bernard-Soulier
c. Chediak-Higashi

A

a. Glanzmann’s
b. Bernard-Soulier
c. Chediak-Higashi

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q
  • Giant platelets that lack** gp Ib/IX/V** receptor
  • Autosomal recessive trait

a. Glanzmann’s
b. Bernard-Soulier
c. Chediak-Higashi

A

b. Bernard-Soulier

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q
  • Normal aggregation with “EAC” and arachidonic acid
  • No aggregation to ristocetin, diminished response to thrombin
  • Prolonged bleeding time
  • Normal CRT, aPTT and PT

a. Glanzmann’s
b. Bernard-Soulier
c. Chediak-Higashi

A

a. Glanzmann’s
b. Bernard-Soulier
c. Chediak-Higashi

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

have the most severe thrombocytopenia among the Inherited Giant Platelet Syndromes

a. Gray platelets
b. Montreal
c. May-Hegglin

A

Montreal platelet syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

no bleeding or other symptoms

a. Mediterranean macrothrombocytopenia
b. Montreal
c. May-Hegglin

A

a. Mediterranean macrothrombocytopenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q
  • autosomal dom
  • Absent or abnormal vWF which is needed for platelets to adhere to collagen in damaged vessels
A

von Willebrand’s Disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Most common hereditary hemorrhagic disorder

a. vWF disease
b. Montreal
c. May-Hegglin

A

a. vWF disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Reduced/lack concentrations
and defective release reaction
of storage granules

a. vWF disease
b. Storage pool disorders
c. May-Hegglin

A

b. Storage pool disorders

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Mutations in genes that encode
proteins involved in intracellular vesicle
trafficking and biogenesis of organelles.

A

Hermansky-Pudlack Syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Characteristics:
1. Oculocutaneous albinism
2. Defective lysosomal function
3. Ceroid-like pigment in macrophages
4. Dense granule deficiency - bleeding

A

Hermansky-Pudlack Syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Mutations resulting to generalized dysfunction involving fusion of cytoplasmic granules

A

Chediak-Higashi
Syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Partial oculocutaneous albinism
2. Frequent pyogenic bacterial infection
3. Hemorrhage
4. Giant lysosomes in all granule-containing cell
5. Dense granule deficiency – bleeding

A

Chediak-Higashi
Syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Dense granules deficiency
* X-linked
* Mutation in WASp
* WASp plays a crucial role in actin
cytoskeleton remodeling

A

Wiskott-Aldrich
Syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q
  1. Microthrombocytopenia (small platelets, low MPV)
  2. Recurrent infections
  3. Eczema
A

Wiskott-Aldrich
Syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Lifelong bleeding (mild) tendencies

A

Gray Platelet Syndrome

17
Q
  1. Large platelets
  2. Gray or blue gray platelets
  3. Thrombocytopenia
  4. BM fibrosis
A

Gray Platelet Syndrome

18
Q

A rare disorder in which both a-granules and dense granules are deficient

A

alpha-Dense Storage Pool Deficiency

19
Q

Inherited deficiency of
cyclooxygenase or thromboxanesynthetase

A

Aspirin-Like Effects

20
Q

Platelets have normal granules but defective release

Thromboxane A2 is required for storage
granule secretion

A

Thromboxane Pathway Disorders:
Aspirin-Like Effects

21
Q

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

A

Scott Syndrome

22
Q

platelets are always in an
“activated” state

A

Stormorken Syndrome

23
Q

antibiotic that block receptor-agonists reaction, inhibit calcium influx

a.B lactams
b. nitrofurantoin

A

a. B lactams

24
Q

Platelets have abnormal shapes,
decreased procoagulant activity,
and a decreased number of
secretory granules

a. Myeloproliferative neoplasm
b. Multiple Myeloma
c. Cardiopulnonary bypass surgery

A

a. Myeloproliferative neoplasm

25
Q

Platelet dysfunction caused by
coating of platelet membranes by
paraprotein

a. Myeloproliferative neoplasm
b. Multiple Myeloma
c. Cardiopulnonary bypass surgery

A

b. Multiple Myeloma

26
Q
  • increased sensitivity to platelet agonists.

a. Sticky platelet
b. spontaneouss aggregation
c. exhausted

A

b. spontaneouss aggregation

Hematology 2 (4 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions