qualitatibe platelet disorder

Question 1

5 platelet qualitative disorder

Answer 1

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

Question 2

platelet adhesion defect

1.bernard soulier syndrome

Answer 2

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)

Question 3

Bernard Soulier’s test result

Answer 3

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

Question 4

platelet adhesion defect

2. Von Willebrand disease

Answer 4

absent/abnormal vwf

Question 5

VWF TEST RESULT

Answer 5

same as BERNAD SOULIER but positve to VWF agtest

Question 6

platelet aggregation defects

1.glanzmannd thrombasthenia

Answer 6

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

Question 7

platelet aggregation defects

2. aspirin ingestion

Answer 7

cyclooxygenase

Question 8

aspirin ingestion

pathway inhibitor-

Answer 8

cause a defect in PLT aggregation

Question 9

Platelet secretion defects

1. gray platelet syndrome

Answer 9

absence of alpha granule
-Platelets appear in wright stained smear

Question 10

Platelet secretion defects

1. storagepool disorder

Answer 10

diminished delta granules or dense bodies occur in inherited disease

Question 11

Platelet secretion defects

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

Answer 11

characterized by enlarged lysosomal vesicles in blood cells

Question 12

Platelet secretion defects

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

Answer 12

autosomal recessive albanism

Question 13

Platelet secretion defects

1. storage pool disorder–1– wiskott ALDRICH syndrome

Answer 13

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

Question 14

Giant platelet disorder

Answer 14

autoimmune disorder

Question 15

immune thrombocytopenic purpura

Answer 15

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

Question 16

caused by glycoprotein abnormalities

Answer 16

bernard soulier syndrome

Question 17

caused by alpha granule defect

Answer 17

gray platelet syndrome

Question 18

characterized by abnormal neutrophil inclusion

Answer 18

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

Question 19

GIANT PLATELET DISORDER

2. SCOTT SYNDROME

Answer 19

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

Question 20

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

Answer 20

RESULTING IN IMPAIRED FORMATION IS SCOTT SYNDROME

Question 21

PHOSPHATIDYLSERINE (PS) -

Answer 21

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

Question 22

VASCULAR DISORDER

Answer 22

*HEREDITARY VASCULAR DISORDERS
*ACQUIRED VASCULAR DISORDER

Question 23

VASCULAR DISORDER

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

Answer 23

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

Question 24

VASCULAR DISORDER

2. EHLER-DANLOS SYNDROME

Answer 24

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

Question 25

ACQUIRED VASCULAR DISORDER 1. HENOCH PURPURA

Answer 25

VASCULITIS MEDIATED BY IMMUNE COMPLEXES CONTAINING IgA antibodies

Question 26

ACQUIRED VASCULAR DISORDER 2. senile purpura

Answer 26

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

Question 27

ACQUIRED VASCULAR DISORDER 3. scurvy

Answer 27

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

Question 28

ACQUIRED VASCULAR DISORDER 4. infectious

Answer 28

meningococcemia, rickettsial infections, staphylococcal infections (Endotoxins)