Platelet and Coagulation Disorders

Qualitative platelet disorder caused by lack of functional GP Ib/IX/V resulting in abnormal platelet adhesion

Bernard-Soulier Syndrome

Qualitative platelet disorder caused by deficiency in GP IIb/IIIa resulting in abnormal platelet aggregation

Glanzmann’s thrombasthenia

Most common inherited bleeding disorder; platelets cant adhere to collagen to from platelet plug

Von Willebrand Disease

Deficiency in Factor VIII

Hemophilia A

Deficiency in Factor IX

Hemophilia B

what is seen in bone marrow hypoplasia

decrease in all blood cell lines

pancytopenia

low counts of all blood cell lines

what is another name for Hemophilia B

Christmas Disease (Factor IX)

What is Factor X (Stuart-Prower Factor) responsible for

activating clot formation

What can liver disease cause

ineffective production of vitamin K dependent factors

lab findings: prolonged bleeding time, thrombocytopenia, giant platelets and abnormal response to ristocetin

Bernard Soulier Syndrome

What factor is also known as Hageman Factor

Factor XII

Lab findings: normal PT and PTT, abnormal plt aggregation except with ristocetin

Glanzmann Thrombasthenia

What can be visibly associated with thrombocytopenia

petechia

What releases Von Willebrand Factor

platelets and endothelial cells

what is lupus anticoagulant

immunoglobulin that acts as a coagulation inhibitor

Lab findings: prolonged PT/PTT, decreased fibrinogen, decreased platelet count, increase in platelet degradation products

Disseminated intravascular coagulation (DIC)

caused by a deficiency in ADAMTS13 Enzyme; increased platelet activation and aggregation resulting in thrombocytopenia; PT and PTT normal

Thrombotic thrombocytopenic Purpura (TTP)

What tests will show a Factor XIII deficiency

Factor XIII assay and 5m urea

Disorder characterized by increased platelet count but abnormal platelet function

Essential Thrombocythemia

Deficiency of this factor is often related to increased bleeding after major surgeries such as dental extraction

Factor XI (hemophilia C)

Clotting and bleeding disorder, often observed in severe sepsis and septic shock; excessive clotting and fibrinolysis

DIC

what is used to aid in diagnosis of BSS

flow cytometry

what is the normal bleeding time

1-7 minutes

Lab findings: decreased platelet count, increased megakaryocytes in bone marrow, often seen in children 2-4 years 1-3 weeks following an infection

immune (idiopathic) thrombocytopenic purpura (ITP)

What test is used to measure titer and activity of inhibitor present

Bethesda assay

what causes platelet satellitosis (satellitism)

antibody directed against platelet membrane

what is dysfibrinogenemia

normal quantities of fibrinogen but abnormal function

What does aspirin inhibit

platelet aggregation by inhibiting cyclooxygenase

What does normal platelet adhesion depend on

Glycoprotein Ib and VWF

What are storage pool deficiencies defects of

platelet granules

Which disorder is associated with neurological problems

TTP

What defect characterizes gray’s syndrome

alpha granule defect