Page 566 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

Disorders of Hemostasis and

28 Trombosis: Blood Coagulation

CHAPTER Factors, Hypercoagulable State, and

Anticoagulant T erapy

KEY TERMS

antithrom bin factor V R506Q (Leiden) protein C

antiphospholipid syndrom e brinolysis protein S
Christm as disease hem ophilia A purpura

contact group hem ophilia B throm bin
dissem inated intravascular hypercoagulable state throm bophilia

coagulation (DIC) lupus anticoagulant

LEARNING OUTCOMES

Introduction ■ Detail how platelets contribute to hypercoagulation.

■ Describe the relationship between hemostasis and blood coagulation. ■ Compare the molecular and functional assays for diagnosis of anti-
thrombin, protein C and protein S abnormalities.
Disorders of Defective Production ■ Describe how activated protein C contributes to thrombophilia.

■ Compare the mechanisms of vitamin K, severe liver disease, and ■ Describe the activity of blood coagulation factors in increasing the
renal disease on defective blood coagulation factor production. tendency toward thrombosis.

■ Compare and contrast the X-linked disorders of secondary hemosta- ■ Explain the characteristic and laboratory ndings in antiphospholipid
sis: factor VIII and factor IX de ciencies. syndrome.

Describe the etiology, pathophysiology, and laboratory testing for ■ Describe the relationship between impaired brinolysis and protein

congenital and acquired von Willebrand’s disorder. C, antithrombin, and plasminogen.

■ Compare the laboratory ndings for factor VIII and factor IX de cien- ■ Describe the laboratory assessments that illustrate the condition of
cies and von Willebrand’s disease. hypercoagulation.

Give examples of uncommon coagulation factor de ciencies and

describe conditions that contribute to the defective production of Anticoagulant Therapy

blood coagulation factors. ■ Discuss various categories of anticoagulant therapy.

Disorders of Destruction and Consumption Case study

■ Compare primary and secondary brinolysis. Analyze the patient history, clinical signs and symptoms, and labo-

■ Explain the conditions and laboratory ndings in conditions of dis- ratory data for the stated case study, answer the related critical
seminated intravascular coagulation (DIC) and brinolysis.
thinking questions, and decide the most likely diagnosis.

The hypercoagulable state (thrombophilia)

■ De ne the term thrombophilia. NOTE:

■ Name and compare primary states of hypercoagulability.
■ indicates MLT and MLS core content
■ Explain the role of vascular damage and blood ow in the hyperco- indicates MLT (optional) and MLS advanced content

agulable state.

IN RODUC ION activities, the c otting actors are initiate to or the brin

c ot. Fibrin or ation can occur i the activity o various actors
Vascu ar res onse an ate et ug or ation are res on- is at east 30% to 40% o nor a . B ee ing an e ective brin

sib e or the initia hases o he ostasis. Subsequent to these c ot or ation are requent y re ate to a coagu ation actor.

550