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Flashcards in qualitatibe platelet disorder Deck (28)
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1
Q

5 platelet qualitative disorder

A
  1. platelet adhesion defects
  2. platelet aggregation defect
  3. platelet secretion defects
  4. giant platelet disorder
  5. vascular disorder
2
Q

platelet adhesion defect

1.bernard soulier syndrome

A

autosomal recessive trait characterized by the absence of GPIB (Receptor of VWF
-characterized by defective platelet adhesion
-detected in vitro aggregation test of platelets (using an aggregometer)

3
Q

Bernard Soulier’s test result

A

plt aggregation is normal with ADP, epinephrine, and collagen but abnormal with ristocetin

4
Q

platelet adhesion defect

  1. Von Willebrand disease
A

absent/abnormal vwf

5
Q

VWF TEST RESULT

A

same as BERNAD SOULIER but positve to VWF agtest

6
Q

platelet aggregation defects

1.glanzmannd thrombasthenia

A

-an autosomal recessive trait
-decreased for the absence of GP/IIB IIA
(Receptor for fibrinogen)
-aggregation test result -normal response only to ristocetin

7
Q

platelet aggregation defects

  1. aspirin ingestion
A

cyclooxygenase

8
Q

aspirin ingestion

pathway inhibitor-

A

cause a defect in PLT aggregation

9
Q

Platelet secretion defects

  1. gray platelet syndrome
A

absence of alpha granule
-Platelets appear in wright stained smear

10
Q

Platelet secretion defects

  1. storagepool disorder
A

diminished delta granules or dense bodies occur in inherited disease

11
Q

Platelet secretion defects

  1. Gray platelet syndrome pool disorder–1– CHEDIAK higashi syndrome
A

characterized by enlarged lysosomal vesicles in blood cells

12
Q

Platelet secretion defects

  1. Gray platelet syndrome pool disorder–1– HERMANSKY PUDLAK syndrome
A

autosomal recessive albanism

13
Q

Platelet secretion defects

  1. storage pool disorder–1– wiskott ALDRICH syndrome
A

x linked recessive disease characterized by a triad of eczema immune deficiency and thrombocytopenia

14
Q

Giant platelet disorder

A

autoimmune disorder

15
Q

immune thrombocytopenic purpura

A

characterized by low platelet count, but high MPV (mean platelet volume)

16
Q

caused by glycoprotein abnormalities

A

bernard soulier syndrome

17
Q

caused by alpha granule defect

A

gray platelet syndrome

18
Q

characterized by abnormal neutrophil inclusion

A

may hegglin anomaly (MYH9 GENE) giant platelets , dohle bodies in WBC

19
Q

GIANT PLATELET DISORDER

  1. SCOTT SYNDROME
A

IS A RARE CONGENITAL BLEEDING DISORDER THAT IS DUE TO A FEDECT IN A PLATELET MECHANISM REQUIRED FOR BLOOD COAGULATION

20
Q

THE MECHANISM FOR TRANSLOCATING PS (BINDING SITE) TO THE PLATELET MEMBRANE IS DEFECTIVE

A

RESULTING IN IMPAIRED FORMATION IS SCOTT SYNDROME

21
Q

PHOSPHATIDYLSERINE (PS) -

A

PROVIDES BINDING SITE FOR PLASMA PROTEINS THAT ARE INVOLVED IN THE CONVERSION OF PROTHROMBIN TO THROMBIN SUCH AS FACTOR VIIIA (8A) - IX (9) (TENASE) AND FACTOR VA(5a) -XA (10a) (PROTHRBINASE)
-scott syndrome

22
Q

VASCULAR DISORDER

A

*HEREDITARY VASCULAR DISORDERS
*ACQUIRED VASCULAR DISORDER

23
Q

VASCULAR DISORDER

  1. HEREDITARY HEMORRHAGIC TELANGECTASIA ALSO KNOWN AS RENDELL-OSLER - WEBER -SYNDROME
A

-PROBLEM IN ELASTIN
-MISSING ELASTIN IN THE SORROUNDING STROMA OF THE BLOOD VESSEL
-CHARACTERIZED BY NORMAL BLEEDING TIME AND INCREASED CAPILLARY FRAGILITY

24
Q

VASCULAR DISORDER

  1. EHLER-DANLOS SYNDROME
A

-XLINKED ASCRIBED BY DEFECT IN COLLAGEN PRODUCTION
-PROLONGED BLEEDING TIME AND CAPILLARY FRAGILITY

25
Q

ACQUIRED VASCULAR DISORDER

  1. HENOCH PURPURA
A

VASCULITIS MEDIATED BY IMMUNE COMPLEXES CONTAINING IgA antibodies

26
Q

ACQUIRED VASCULAR DISORDER

  1. senile purpura
A

-common in an elderly man
-lack of collagen support
for small blood vessels and loss of subcutaneous fat and elastic fiber

27
Q

ACQUIRED VASCULAR DISORDER

  1. scurvy
A

-ascorbic acid (Vit. c deficiency)
-result in decreased synthesis of collagen and hyaluronic acid

28
Q

ACQUIRED VASCULAR DISORDER

  1. infectious
A

meningococcemia, rickettsial infections, staphylococcal infections (Endotoxins)