3

Question 1

DEFICIENCY OF GRANULES

1. structural defect in beta granules w/ corresponding abnormal aggregation responses & thrombocytopenia
   - characterized by _______ & _____ (most pronounced skeletal abnormality)
2. autosomal dominant disorder characterized by abnormal proteolysis & deficiency of alpha granules
   - delayed for _____ of _____
   - excessive amounts of _____ which is a serine protease that helps in conversion of plasminogen to plasmin

Answer 1

1. Thrombocytopenia w/ Absent Radii Syndrome (TAR)
   - severe neonatal thrombocytopenia & extreme hypoplasia of radial bones
2. Quebec Platelet Disorder
   - 12-24hrs mucocutaneous bleeding
   - urokinase-type plasminogen activator

Question 2

DISORDERS OF PLATELET PROCOAGULANT ACTIVITIES

During rest:
1. Located in inner leaflet
2. Located in outer leaflet

During platelet activation:
1. This process happens

Disorders:
1. surface expression of phospatidylserine is decreased (phospatidylserine doesn’t come out)
2. platelets are always in an activated state w/o prior activation
- there’s high ___ levels even there’s no vascular injury
- hence ____ & _____ are consumed entirely
3. Both disorders are due to

Answer 2

During rest:
1. phosphatidylserine (PS) & phosphatidamine (PE)
2. phosphatidylcholine

During platelet activation:
1. Platelet Membrane Phospholipid Scrambling

Disorders:
1. Scott Syndrome
2. Stormorken Syndrome
- calcium levels
- spontaneous aggregation and coagulation factors
3. genetic mutation

Question 3

ACQUIRED DEFECTS OF PLATELET FUNCTION

1. Drug related
   - NSAIDS meaning
   - COX2 inhibitors (2)
   - COX2 inhibitors inhibits the activity of ____ which is precursor of thromboxane a2 which helps vasoconstriction & platelet aggregation
   - other antiplatelet agents (2) that helps prevents clumping of platelet during
   platelet aggregation
   - coats the surface of platelets where there are glycoproteins, there’s no binding w/ fibrinogen which will inhibit platelet aggregation
2. 2 Disorders in paraproteinemias
   - increased protein in blood
   - increased IgM
   - excessive plasma protein lead to
3. Renal disease
   - this leads to decreased thromboxane synthesis, decreased adhesion, decreased
   platelet release, and decreased aggregation
   - exhibited during ____ renal disease only

Answer 3

1. Drug related
   - non- steroidal anti inflammatory drugs
   - naproxen & ibuprofen
   - cyclooxygenase
   - ticlopidine & clopidogrel)
   - dextran

2.
- multiple myeloma
- waldenstrom macroglobulinemia
- hyperviscosity syndrome

3. Renal disease
   - uremia
   - end stagerenal disease only

Question 4

Significant Platelet Levels

1. abnormally low
2. bleeding possible
3. spontaneous bleeding
4. evere spontaneous bleeding

Answer 4

1. <100,000/uL
2. 30,000-50,000/uL
3. <30,000/uL
4. <5,000/uL

Question 5

THROMBOCYTOPENIA: Decreased Production

Megakaryocyte Hypoproliferation in:
1. genetic disorder that affects the blood cells esp. platelets & wbcs
- ____ can only infect neutrophils
- may-hegglin inefects (3)
2. in neonatal thrombocytopenia:
- TORCH stands for
- in-utero exposure to certain drugs

Answer 5

1. May-Hegglin
   - dohle bodies
   - BEN
2. • toxoplasmosis, other agents, rubella, cytomegalovirus, herpes
   • chlorothiazide diuretics & oral hypoglycemic tolbutamide)

Question 6

THROMBOCYTOPENIA: Decreased Production.2

Two other causes in decreased production
1. seen in megaloblastic anemias
2. ________ by abnormal cells

Answer 6

1. Ineffective thrombopoiesis
2. Marrow replacement (infiltration) by abnormal cells

Question 7

THROMBOCYTOPENIA: Blood Transfusion

1. • recipient’s plasma is found to contain
     2.

Answer 7

1. Platelet-containing blood products
   - alloantibodies
2. Massive transfusion

Question 8

THROMBOCYTOPENIA: Increased Destruction or Consumption

Non Immune
1. deficiency of disintegrin & metalloproteinase w/ thrombospondin type 1 motif member
- what is this motif member
- when there’s deficiency of this enzyme/motif me,her, excess platelet adhesion causes ______
2. activation of both coagulation & fibrinolysis due to liberation of thromboplastin substances by damaged or abnormal cells/ epithelial cells
- three bacteria’s that produces toxin
- E.coli release toxin called
3. example of infection that causes decreased platelets

Answer 8

Non Immune
1. Thrombotic Thrombocytopenic Purpura
- ADAMTS-13
- thrombus
2. Disseminated Intravascular Coagulation (DIC)
- E.coli O157:H7, Shiga, or Vero toxin-producing bacteria
- Shiga-like toxin 1 or 2
3. dengue

Question 9

THROMBOCYTOPENIA: Increased Destruction or Consumption

Immune
1. common in females
- presence of ____
- acute ITP common in
- chronic ITP common in
2. Drug Induced, which is called _______
- patient develops IgG
antibody specific for _____
- they gives _____

Answer 9

Immune
1. Immune Thrombocytopenic Purpura
- anti-platelet antibodies of IgG
- children
- adults
2. Heparin-Induced Thrombocytopenia
- heparin-platelet factor 4 complexes
- unfunctionated heparin

Question 10

THROMBOCYTOPENIA:
_____: removal of platelets in circulation
1. seen in ____ & ____

THROMBOCYTOSIS/ THROMBOCYTHEMIA:
1. Primary thrombocytosis are seen in
2. Secondary are seen in ____ (removal of spleen) & _____

Answer 10

THROMBOCYTOPENIA:

Splenic sequestration
1. hypersplenism & splenomegaly

THROMBOCYTOSIS/ THROMBOCYTHEMIA:
1. myeloproliferative disorders
2. splenic mobilization; hemolytic anemia