TESTS FOR AND DISORDERS OF PRIMARY HEMOSTASIS

Question 1

Normal platelet count

Answer 1

150 - 450 x 10^9/L

Question 2

Is the most common cause of clinically important bleeding

Answer 2

Plt ct of <100,000 uL

Question 3

Reference method for plt counting

Answer 3

Phase contrast microscope metho d

Question 4

EDTA whole blood is diluted ___ : ___ with _% ____ to lyse the nonnucleated RBCs

Answer 4

1:100 ; 1% ammonium oxalate

Question 5

Platelets are counted in the ___ ____ ____ of the ___ ___ ___ (1mm2) of he hemocytometer

Answer 5

25 small squares of the central large square

Question 6

The charged hemocytometer is then placed in a moist chamber for ____ mins to allow platelets to settle

Answer 6

15 mins

Question 7

Difference between the total cells counted on each side should be

Answer 7

<10%

Question 8

Percentage difference formula

Answer 8

(V1-V2) / (V1 + V2 / 2) X 100

Question 9

Platelet count formula

Answer 9

Cells counted x df / area (mm2) x depth (0.1)

Question 10

A normal (wedge) blood smear should demonstrate approx

Answer 10

7 to 21 cells per 100x field

Question 11

Platelet estimate formula

Answer 11

Average no of plts per field x total RBC count / 200 RBCs per field

Question 12

Average number of RBCs per oil immersion field in the optimal assessment area

Answer 12

200

Question 13

Designed to test platelet-rich plasma

Answer 13

Light-transmittance (optical) platelet aggregometry

Question 14

PRP has a platelet count of

Answer 14

200,000 to 300,000/uL

Question 15

Examples of aggregating reagents

Answer 15

Adenosine diphosphate
Collagen
Epinephrine
Ristocetin

Question 16

Examples of disorders with NORMAL response of ADP, collagen, epinephrine

Answer 16

Bernard soulier syndrone
Von willerbrand disease

Question 17

Examples of disorders with ABNORMAL response of ADP, collagen, epinephrine

Answer 17

GLANZMANN THROMBASTHENIA

Question 18

Example of disorder with NORMAL response of Ristocetin

Answer 18

Glanzmann’s thrombasthenia

Question 19

Example of disorder with ABNORMAL response of Ristocetin

Answer 19

BERNARD SOULIER SYNDROME
VON WILLEBRAND DISEASE

Question 20

Example of disorder with ABNORMAL response of Ristocetin

Answer 20

BERNARD SOULIER SYNDROME
VON WILLEBRAND DISEASE

Question 21

Measures platelet aggregation and ATP release (from dense granules)

Answer 21

Platelet lumiaggregometry

Question 22

ATP oxidizes a firefly-derived ______ reagent to produce cold chemiluminescene proportional to the ATP concentration

Answer 22

Luciferin-luciferase reagent

Question 23

Normal results in vascular disorder

Answer 23

Platelet cont
Platelet function test
Coagulation tests

Question 24

Abnormal results in vascular disorders

Answer 24

Bleeding time
Rample-Leed test

Question 25

Vascular disorders

Answer 25

Hereditary hemorrhagic telangiectasia Helpers-Danilo’s Syndrome

Question 26

Aka Rendu-Osler-Weber syndrome

Answer 26

Hereditary hemorrhagic telangiectasia

Question 27

Aka cutis hyperelastica

Answer 27

Ehlers-Danilo’s syndrome

Question 28

Most common inherited vascular bleeding disorder ; autosomal dominant ; with localized dilation of capillary walls

Answer 28

Hereditary hemorrhagic telangiectasia

Question 29

Vascular disorder characterized by hyperextensible skin, hypermobile joints, joint laxity, fragile tissues, hematoma formation ; autosomal dominant

Answer 29

Ehlers-Danilo’s syndrome

Question 30

Vascular disorder Most commonly seen in children ; most probably cause by immunologic damage to the endothelial cells ; characterized by gastrointestinal intestinal hemorrhage and joint swelling

Answer 30

Henoch schonlein purpura

Question 31

Aka allergic purpura or non thrombocytopenia purpura

Answer 31

Henoch schonlein purpura

Question 32

Vascular disorder characterized by defects in the synthesis of collagen and Hyaluronic acid ; vit C deficiency

Answer 32

Scurvy

Question 33

Aka secondary thrombocytosis

Answer 33

Reactive thrombocytosis

Question 34

Examples of cases with reactive thrombocytosis

Answer 34

Recovery from splenectomy Acute blood loss Major surgery

Question 35

Characterized by MODERATELY increased platelet count

Answer 35

Reactive thrombocytosis

Question 36

Aka primary thrombocytosis

Answer 36

Autonomous thrombocytosis

Question 37

Characterized by MARKEDLY increased platelet count

Answer 37

Autonomous thrombocytosis

Question 38

Examples of cases with autonomous thrombocytosis

Answer 38

Essential thrombocythemia Chronic myelogenous leukemia Polycythemia Vera Primary myelofibrosis

Question 39

Examples of cases with autonomous thrombocytosis

Answer 39

Essential thrombocythemia Chronic myelogenous leukemia Polycythemia Vera Primary myelofibrosis

Question 40

Examples of cases with autonomous thrombocytosis

Answer 40

Essential thrombocythemia Chronic myelogenous leukemia Polycythemia Vera Primary myelofibrosis

Question 41

Group of malignant neoplasms ; described as clonal proliferations of bone marrow stem cells ; characterized by elevations in or more myeloid cell types in the peripheral blood

Answer 41

Myeloproliferative neoplasm

Question 42

Uncontrolled proliferation of bone marrow Megakaryocytes ; plt count exceeding 1 million / uL

Answer 42

Essential thrombocythemia

Question 43

Most common congenital bleeding disorder

Answer 43

Von willebrand’s disease

Question 44

Primary platelet surface receptor for VWF

Answer 44

GP Ib/IX/V

Question 45

IMPORTANT IN PLATELET ADHESION

Answer 45

GP Ib/IX/V VWF

Question 46

Low factor VIII , normal PT , prolonged APTT , Normal aggregation ACE ; abnormal aggregation in Ristocetin

Answer 46

VWD

Question 47

Normal or increased bleeding time ; mild bleeding tendency ; petechiae none ; MOST COMMON vWD

Answer 47

Type 1

Question 48

Rarest type of VWD

Answer 48

Type III

Question 49

Platelet adhesion disorders

Answer 49

Bernard soulier VWD

Question 50

Platelet aggregation disorders

Answer 50

Glanzmann’s thrombasthenia Hereditary afibrinogenemia

Question 51

Deficiency of GP IIb/IIIa

Answer 51

Glanzmann’s thrombasthenia

Question 52

Very prolonged bleeding time ; abnormal clot retraction ; abnormal aggregation response in ACE ; normal aggregation response in Ristocetin

Answer 52

Glanzmann’s thrombasthenia

Question 53

Process wherein platelets adhere to other platelets

Answer 53

Platelet aggregation

Question 54

Important in platelet aggregation

Answer 54

GP IIb/IIIa Fibrinogen

Question 55

Thromboxane pathway disorders ; Cyclooxygenase and Thromboxane synthetase deficiency

Answer 55

Platelet secretion disorder

Question 56

Alpha and dense granule deficiency

Answer 56

Pool storage diseases

Question 57

Alpha granule deficiency ; autosomal recessive

Answer 57

Gray platelet syndrome

Question 58

Characterized by plasma levels of platelet factor 4 and B-thromboglobulin is increased

Answer 58

Gray platelet syndrome

Question 59

Autosomal dominant ; bleeding disorder that results from multimerin deficiency

Answer 59

Quebec platelet disorder

Question 60

A protein stored in alpha granules associated with Quebec platelet disorder

Answer 60

Multimerin

Question 61

Dense granules deficiency ; oculocutaneous albinism ; normal plt

Answer 61

Hermansky-Pudlak syndrome

Question 62

Dense granules deficiency ; partial albinism ; low plt ct

Answer 62

Chediak-Higashi syndrome

Question 63

Dense granules deficiency; no albinism ; low plt ct

Answer 63

Wiskott-Aldrich syndrome

Question 64

Dense granules deficiency; no albinism ; normal plt ct

Answer 64

TAR syndrome

Question 65

All of the dense granules deficiencies are autosomal ___ except for Wiskott-Aldrich syndrome which is ____ recessive

Answer 65

Autosomal recessive ; x-linked recessive

Question 66

Plt count during infancy and within 1 year of birth in the case of Wiskott-Aldrich syndrome

Answer 66

Low in infancy and normal within 1 year of birth

Question 67

Most common acquired bleeding disorder

Answer 67

Trauma-induced coagulopathy