Hematology Exam 6 Flashcards
Vascular integrity and platelets forming a primary, temporary plug are a part of ________ coagulation.
Primary coagulation
Plasma coagulation factors are a part of _________ coagulation
Secondary coagulation
Blood fluidity is dependent on the fibrin lysing system and _______ to coagulation in order to prevent excess clot formation
Inhibitors
What are the other names for Factor III?
- Tissue factor
- Tissue thromboplastin
Rare condition producing abnormal connective tissue and poor support
Telangiectasia
Is telangiectasia an inherited or acquired vascular disorder?
Inherited
Type of vascular disorders that may damage endothelial cells or connective tissue
Acquired vascular disorders
Blood pressure cuff at 80mmHg for 5 minutes then taken off to count petechiae present in a 3 square centimeter area
Tourniquet test
Normal platelet range
150,000-450,000/mm3
What percentage of platelets are reserved in the spleen?
33%
Maturation of megakaryocytes include what 3 stages?
- Megakaryoblast
- Promegakaryocyte
- Megakaryocyte
What influences the multi-potential stem cell to produce megakaryocytes?
CSF-MEG and thrombopoietin
66% of platelets cirtculate how many days before the spleen destroys them?
5-10 days
New terminology for the peripheral zone of a platelet
Platelet plasma membrane
New terminology for the structural zone of a platelet
Platelet cytoskeleton
What is the bilayer plasma membrane of a platelet composed of?
-Phospholipids and cholesterol
Purpose of cholesterol in the bilayer of the plasma membrane of a platelet
Stability, fluidity, transmembranous passageway
What is the fluffy outer coat that contains factors for adhesion and aggregation on platelets known as?
Glycocalyx
Glycoprotein that allow platelet to adhere to collagen
GP Ia/IIa
Glycoprotein that allows platelet to bind to vWF
GP Ib/IX/V
Glycoprotein that allows binding of fibrinogen to vWF
GP IIb/IIIa
Part of the platelet that acts as a barrier and regulates metabolic function
Plasma membrane
(aka Peripheral zone)
This section of the platelet is where the “inside/outside” response occurs
Platelet cytoskeleton
(aka structural zone)
What are the mcirofilaments in the platelet cytoskeleton composed of?
Actin
Purpose of actin in the platelet cytoskeleton
Anchors the plasma membrane glycoproteins
Section of the platelet that enables it to store additional quantities of hemostatic proteins found in the glycocalyx
Surface Connected Canalicular System (SCCS)
This part of the platelet sequesters calcium and bears enzymes that support platelet activation
Dense tubular system
Enzymes found in the dense tubular system (3)
- Cyclooxygenase
- Thromboxane synthetase
- Phospholipase A2
What section of the platelet is known as the control center for platelet activation?
Dense tubular system
What are the 2 major types of granules in platelets?
- Dense
- Alpha
Which platelet granules contains ADP, ATP and Ca2+?
Dense granules
These platelet granules contain Factor V, Fibrinogen, vWF, and plasma coagulation activation factors such as HMWK
Alpha granules
Which platelet granules will migrate to the plasma membrane and release their contents directly into plasma upon activation?
Dense granules
Alpha platelet granules will fuse to which part of a platelet when activated and release their contents?
SCCS (Surface connected cannalicular system)
4 Main functions of platelets
- Adhesion
- Shape change
- Aggregation
- Secretion
What stimulates the formation of thromboxane A2?
Arachidonic acid
A platelet count below what number will cause spontaneous bleeding?
Less than or equal to 10,000/µL
Which platelet test involves a shallow cut made on the forearm and then time is measured regarding the amount of time it takes for the site to stop bleeding when constant pressure in applied via a BP cuff at 40mmHg?
Bleeding time
Examples of primary disorders that cause a quantitative defect resulting in a high platelet count (4)
Myeloproliferative disorders such as:
- Polycythemia vera
- Chronic myeolcytic leukemia
- Myelofibrosis with myeloid metaplasia
- Essential thrombocythemia
Examples of secondary disorders that cause a quantitative defect resulting in a high platelet count (3)
- Inflammation
- Iron deficiency
- Acute blood loss
Aplastic anemia, megaloblastic anemia, marrow infiltraion by malignant cells, and chemotherapy would all cause quantitative platelet defects resulting is ___________ (low/high) platelet counts.
LOW platelet counts
- Increased platelet destruction due to an autoantibody to platelets
- Fairly common
- May be acute or chronic
- Acute is seen in children following infection
- Chronic is often seen in women of child-bearing years
Idiopathic Thrombocytopenia Purpura (ITP)
Quantitative platelet defect:
- Ages 2-6 years
- Platelet count <20,000/uL
- Petechiae are common
- Following a viral infection such as rubella, chickenpox, CoV-19
- Antibody produced against viral antigens bind to the platelet surgace causing destruction of platelet
Acute ITP
TRUE or FALSE:
Acute ITP is typically self-limiting and will resolve itself
TRUE
Quantitative platelet defect:
- Ages 20-40 years old
- Onset is gradual
- Platelet count 30,000-80,000/uL
- More common in females
Chronic ITP
Heparin and quinidine are drugs that cause increased platelet destruction due to actings like ______ which eleicit an antibody response
(Think immunology)
Haptens
Platelets contain more of which major type of granule?
Alpha granules (50-80 per platelet)
Which glycoprotein will vWF bind to on a platelets surface to make the platelet adhere reversibly to the vessel wall?
Glycoprotein Ib
Exposure to collagen and ADP being secreted will cause platelets to do what?
Platelets will change shape from discoid to a spiny sphere with pseudopods
For platelet aggregation, what glycoprotein will fibrinogen bind to?
Glycoprotein IIb-IIIa
What is produced during secondary aggregation?
An irreversible plug
What is produced by plasma clotting factors in order to reinforce the irreversible platelet plug to stop bleeding?
Fibrin
3 different platelet tests
- Platelet count
- Bleeding time
- Platelet aggregation
Which platelet test checks platelet count as well as vascular integrity?
Bleeding time
What are the commonly usecd aggregating agents in platelet aggregation studies?
Epinephrine, ADP, Collagen, and Ristocetin
Antibodies produced against viral antigens (chickenpox, rubells, etc.) bind to the platelet surface causing the platelet to be destroyed
Acute ITP
What 2 drugs act as haptens and elicit an antibody response creating an antigen-antibody complex that attaches to the platelet surface causing the platelet to be removed from circulation?
Heparin and Quinidine
What are the 3 most common causees of increaased platelet destruction due to non-immune destruction?
- Thrombotic Thrombocytopenic Purpura (TTP)
- Hemolytic Uremic Syndrome (HUS)
- Disseminated Intravascular Coagulation (DIC)
Best way to tell the difference between ITP and TTP?
Neurological symptoms are seen with TTP and not with ITP
How is TTP treated?
Plasma exchange
- Remove ULVWF
- Replace with fresh frozen plasma (FFP) or cryoprecipitate poor plasma
As well as corticosteroids
Which platelet disorder is missing ADAMTS-13?
Thrombotic thrombocytopenic purpura
Purpose of ADAMTS-13
Cleaves the ULVWF (ultra-large vonWillebrand Factor) multimers into smaller vWF multimers
In the absence of ADAMTS-13, ULVWF will bind to which platelet glycoproteins?
GP Ib and GP IIb/IIIa
ULVWF binding to GP Ib and GP IIb/IIIa causes (increased/decreased) adhesion and aggregation which forms hyaline thrombi
Increased
Name the platelet disorder:
- Decreased platelet count
- Schistocytes seen on PB smear
- Decreased haptoglobin
- Increased LDH and Bilirubin
- Normal PT, PTT, Fibrinogen, FSPs, and D-Dimer
TTP
This is found only in children following an infection, typically with either Salmonella or E coli.
HUS
Hemolytic Uremic Syndrome
How are HUS and TTP differentiated?
TTP has neurologic symptoms as well as usually having a more severe thrombocytopenia
In HUS the platelet consumption is usually occurring in the kidneys
Clotting occurs throughout the whole body.
Increased PT, PTT, FSP, and D-Dimer
Decreased platelet count and Fibrinogen
DIC
Disseminated Intravascular Coagulation
Types of qualtitative platelet defects (3)
- Adhesion
- Aggregation
- Secretion
Most common adhesion defects (2)
- Von Willebrand’s Disease
- Bernard-Soulier Disease
Defective glycoprotein Ib causing an inability to bind to vWF. Patients exhibit hemorrhage characteristics (expanding large hematomas)
Bernard-Soulier Disease
Low, absent, or defective VIII:vWF causing defective platelet adhesion
Von Willebrand’s Disease
Aggregation platelet defect due to a defective glycoprotein IIb/IIIa. Causes aa inability to bind fibrinogen and form bridges for aggregation
Glanzmann’s