Lesson 3 Flashcards
Deficiency of Granules (Storage Pool Disorders)
_____: structural defect in beta granules w/ corresponding abnormal aggregation responses & thrombocytopenia
1. characterized by _____ & _____ (most pronounced skeletal abnormality), _____, and other skeletal abnormalities
_____: autosomal dominant disorder characterized by abnormal proteolysis & deficiency of alpha granules
1. delayed (____hrs) mucocutaneous bleeding
2. excessive amounts of ____ = serine protease that helps in conversion of plasminogen into plasmin
Thrombocytopenia w/ Absent Radii Syndrome
1. severe neonatal thrombocytopenia & congenital absence or
2. extreme hypoplasia of radial bones (most pronounced skeletal abnormality), cardiac
Quebec Platelet Disorder
1. 12-24hrs
2. urokinase-type plasminogen activator
DISORDERS OF PLATELET PROCOAGULANT ACTIVITIES
In resting platelets, _______ & _______ are located predominantly on inner leaflet while ______ has are located on outer leaflet
1. surface expression of phospatidylserine is decreased
2. platelets are always in an activated state w/o prior activation
inner = phosphatidylserine & phosphatidamine
outer = phosphatidylcholine
1. Scott Syndrome
2. Stormorken Syndrome
ACQUIRED DEFECTS OF PLATELET FUNCTION
Drug related
1. ________ & other COX2 inhibitors
2. inhibits the activity cyclooxygenase
- examples
3. other platelet agents (2) = prevents clumping of platelet during platelet aggregation
4. coats the surface of platelets where there are glycoproteins
____: excessive plasma protein lead to hyperviscosity syndrome
1. increased protein in blood
2. increased IgM
Renal Disease
1. leads to decreased thromboxane synthesis, decreased adhesion, decreased platelet release, and decreased aggregation
Drug related
1. NSAIDS (non- steroidal anti inflammatory drugs)
2. COX-2 inhibitors
- naproxen & ibuprofen
3. ticlopidine & clopidogrel
4. dextran
Paraproteinemias
1. multiple myeloma
2. waldenstrom macroglobulinemia
Renal Disease
1. uremia
Significant Platelet Levels
abnormally low
bleeding possible
spontaneous bleeding
severe spontaneous bleeding
<100,000/uL
30,000-50,000/uL
<30,000/uL
<5,000/uL
Disorders in Quantitative Platelet Disorders: Decreased Production
1. genetic disorder that affects the blood cells esp. platelets & wbcs; can only infect baso, eso, neutrophil (BEN)
2. TORCH & in-utero exposure to certain drugs
3. seen in megaloblastic anemias
- May-Hegglin
- Neonatal Thrombocytopenia
- Ineffective thrombopoiesis
Disorders in Quantitative Platelet Disorders: Blood Transfusion
1. occurs after transfusion of platelet-containing blood products
- recipient’s plasma is found to contain ____
2. frequent blood transfusion can cause overload; excess iron can be accumulated inside bone marrow
- Platelet-containing blood products
- alloantibodies - Massive transfusion
Disorders in Quantitative Platelet Disorders: Increased Destruction or Consumption
Non-immune
1. deficiency of disintegrin & metalloproteinase w/ thrombospondin type 1 motif member (ADAMTS-13)
2. activation of both coagulation & fibrinolysis due to liberation of thromboplastin substances by damaged or abnormal cells/ epithelial cells
3. mild febrile illnesses, certain immunizations, & gastrointestinal disturbances (E.coli O157:H7, Shiga, or Vero toxin-producing bacteria)
4. dengue causing decreased platelets
Immune
1. presence of anti-platelet antibodies of IgG type; common in females (<15)
2. patient develops IgG antibody specific for heparin-platelet factor 4 complexes
Non-immune
1. Thrombotic Thrombocytopenic Purpura
2. Disseminated Intravascular Coagulation
3. Hemolytic Uremic Syndrome
4. Infections
Immune
1. Immune Thrombocytopenic Purpura
2. Heparin-Induced Thrombocytopenia
Thrombocytosis/ Thrombocythemia: increased level of platelets
1. seen in myeloproliferative disorders (bone marrow); e.g. leukemia
2. seen in splenic mobilization (removal of spleen) and in hemolytic anemia
- Primary thrombocytosis
- Secondary/ Reactive thrombocytosis
