Platelet substances, Coagulation cascade, Platelet defects (raw/unedited)

➔ True or false: Platelet secretes up to 300 substances True

➔ Platelet factor which is a lipoprotein required in 2 steps of coagulation

process PF3

➔ Glycoprotein that aids in ADP-induced platelet aggregation PF4

➔ Glycoprotein that inhibits the effects of heparin PF4

➔ Alpha granules that promote coagulation HMWK, Fibrinogen, Factor V,

VWF

➔ Dense granules that promote aggregation ADP, Calcium

➔ Alpha granules that promote aggregation PF4, Thrombosponsin

➔ Alpha granules that promote vasoconstriction Thromboxane A2

precursors

➔ Dense granules that promote vasoconstriction Serotonin

➔ Alpha granules that promote vascular repair PDGF, Beta

Thromboglobulin

➔ Alpha granule that participate in fibrinolysis Plasminogen

➔ Alpha granules that prevents complement activation C1 esterase

inhibitor

➔ change the shape of the platelets for compatibility of aggregation ADP

➔ Important dense granule substance for coagulation system Calcium

➔ A strong mitogen that promotes growth of fibroblast PDGF, beta

thromboglobulin

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➔ cells that secretes collagen Fibroblast

➔ Inhibits fibrinolysis A2 antiplasmin

➔ Degradation product of fibrin D-Dimer

➔ Cells that produce VWF endothelial cells, platelets, monocyte

➔ What organ produces fibrinogen Liver

➔ Substance that initiates the coagulation pathway HMWK

➔ Where is HMWK produced? Platelets

➔ Reagent for testing of clotting of aPTT and PT PF3

➔ Coagulation factor that is mostly with VWF Factor VIII

➔ Required coagulation factor to convert prothrombin to thrombin Va,

Xa

➔ Coagulation factor that converts fibrinogen to fibrin I

➔ Coagulation factor that stabilized the fibrin clot XIII

➔ Extrinsic pathway is always initiated with? Tissue factor

➔ Intrinsic pathway is most commonly initiated with? HMWK

➔ Enumerate in summary the cascade of events in intrinsic pathway

HMWK/PK, XII, XI, IX, X, V, II, I

➔ Enumerate in summary the cascade of events in extrinsic pathway TF, VII,

X, V, II, I

➔ Enumerate in summary the cascade of events in common pathway X, V,

II, I

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➔ Deficiency in platelet surface receptor resulting to problem in adhesion to

collagen Bernard-Soulier syndrome

➔ T/F: Bernard-Soulier syndrome aggregates with Ristocetin False

➔ If there is a problem with platelet adhesion, blood will aggregate

abnormally with? Ristocetin

➔ If there is a normal aggregation but a problem with platelet adhesion, blood

will aggregate normally with? Epinephrine, ADP, Collagen

➔ 2 platelet adhesion defects discussed Bernard-soulier syndrome, Von

willebrand disease

➔ Platelet adhesion defect with Factor VIII deficiency Von Willebrand

Disease

➔ Anti-hemophilic factor VIII

➔ Common sign of Von Willebrand Disease mucocutaneous bleeding

➔ True or false: in von willebrand disease, platelet count and morphology is

normal True

➔ Laboratory findings in Bernard-soulier syndrome Prolonged bleeding time

➔ 3 standard von Willebrand disease tests vWF Antigen assay,

vWF/Ristocetin cofactor assay, Factor VIII activity assay

➔ Treatment options for von Willebrand disease Cryoprecipitate,

Desmopressin acetate

➔ Desmopressin acetate is a synthetic analogue of AVP of and it stimulates

the production of? vWF, Factor VIII

➔ Cryoprecipitate contains what coagulation proteins? vWF, I, VIII, XIII

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➔ 3 coagulation factors that may spark the start of common pathway

VIIa, VIIIa, IXa

➔ vWD with partial quantitative vWF deficiency Type 1

➔ vWD with qualitative vWF deficiency Type 2

➔ vWD with selective deficiency of HMW multimeters Type 2A

➔ The vWF is more susceptible to proteolysis by ADAMTS-13 Type 2A

➔ vWD with increased affinity for platelet glycoprotein Ib/IX Type 2B

➔ vWD with decreased platelet receptor binding Type 2M

➔ vWD with impaired factor VIII binding site Type IIN

➔ vWD also called the Normandy variant Type 2N

➔ vWF is absent or nearly absent on plasma Type 3

➔ synthesized in liver and responsible for cleaving VWF anchored on

endothelial surface ADAMTS-13

➔ Autosomal recessive trait with Gp IIB-IIIa deficiency Glanzmann’s

Thrombasthenia

➔ True or false: abnormal platelet aggregation is reflected by non-

aggregation with Ristocetin False

➔ In-vivo, abnormal aggregation is reflected by a negative result in what

aggregating reagents? ADP, Epinephrine, Collagen

➔ True or false: platelets that has abnormal aggregation function still

aggregate with ristocetin True

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➔ Congenital defects that may lead to dysfunctional plt-plt interaction

Afibrinogenemia, hypofibrinogenemia

➔ Platelet surface receptor responsible for plt-plt interaction Gp IIb-IIIa

➔ Platelet surface receptor responsible for plt-nonplt interaction Gp Ib-IX

➔ Alpha granule disorders Gray platelet syndrome, Quebec platelet syndrome

➔ Dense granule disorders Wiskott-aldrich syndrome, Hermansky-Pudlak

syndrome, Chediak-Higashi syndrome

➔ Beta granule disorder Thrombocytopenia with absent Radii syndrome

➔ Major secretor of collagen fibroblasts

➔ Macrothrombocytes, BT increase, Aggregation decrease, what disorder?

Gray platelet syndrome

➔ Microthrombocytes, thrombocytopenia, recurrent infection and eczema,

what disorder? Wiskott-aldrich syndrome

➔ Macrophage pigment is seen, there is bleeding tendency, albinism

including in eyes, what disorder? Hermansky-Pudlak syndrome

➔ Giant lysosomes are seen, recurrent infection and albinism is observed,

what disorder? Chediak-Higashi

➔ Thrombocytopenia with extreme hypoplasia of radial and other bones

Thrombocytopenia with absent Radii syndrome

➔ Autosomal dominant disorder characterized by delayed mucocutaneous

bleeding Quebec platelet disorder

➔ True or false: Quebec platelet disorder is an autosomal recessive trait

False

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➔ Marker of platelet activation exposed phosphatidylserine

➔ Disorder wherein surface expression of phosphatidylserine is decreased

Scott syndrome

➔ Disorder wherein platelets are always in activated state without proper

activation Stormorken syndrome

➔ Inner leaflet structures in a resting platelet phosphatidylserine,

phosphatidylethanolamine

➔ Outer leaflet structure in a resting platelet phosphatidylcholine

➔ Drugs that may cause platelet defects Aspirin, NSAIDs, Naproxen, Ibuprofen,

Ticlopidine, Clopidogrel, Dextran

➔ Drugs that inhibits TxA2 production resulting to reduced aggregating

property of platelet NSAIDs, Aspirin, Naproxen, Ibuprofen

➔ Drug that inhibits ADP pathway resulting to impaired platelet activation and

aggregation Clopidogrel

➔ To prevent complications due to clot, AMI patients are commonly

prescribed with what drugs? Aspirin, Clopidogrel

➔ Diseases of hyperviscosity syndrome that interferes with platelet function

Multiple Myeloma, Waldenstrom Macroglobulinemia

➔ True or false: Uremia interferes with ALL platelet functions True

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