Thrombotic Disorders (M) Flashcards
What are thrombotic disorders?
These are disorders resulting from decreased lvls of regulatory proteins, defects of coagulation factors and presence of non-specific inhibitors resulting to formation of thrombus
What are the true conditions resulting to thrombosis?
1) Antithrombin deficiency
2) Protein C deficiency
3) Protein S deficiency
4) Activated Protein C Resistance
5) Prothrombin Gene Mutation
6) Systemic Fibrinolysis
7) Antiphospholipid Syndrome
8) Arterial Thrombosis
9) Venous Thromboembolic Disease
10) Disseminated Intravascular Coagulation
Answer the ff questions:
Given disease: Antithrombin Deficiency
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) None
2) Autosomal dominant
3) a. Coagulation regulatory protein deficiency
b. No protein is present to neutralize IIa, IXa, Xa, XIa, XIIa, pre-Kallikrein, and plasmin
c. Qualitative - structural abnormalities of antithrombin
d. Quantitative - low lvls of antithrombin in the circulation
4) a. Associated w/ pregnancy, liver disease, nephrotic syndrome
5) None
6) a. Antithrombin lvls: decreased
b. Antithrombin activity assay: decreased
7) a. Antithrombin concentrate
Answer the ff questions:
Given disease: Protein C deficiency
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) None
2) Autosomal dominant, autosomal recessive
3) a. Coagulation regulatory protein deficiency
b. No enzyme is present to inactivate factor Va and VIIIa
c. Heterozygous - decreased activity and concentration
d. Homozygous - will result to purpura fulminans
4) a. Associated with pregnancy, liver disease, renal disease, vitamin K deficiency, DIC
5) a. Skin necrosis
b. Thrombosis within the dermal vessels
c. Pruritus
6) a. Protein C lvls: decreased
b. Protein C activity assay: decreased
7) a. Protein C concentrate
b. Lifelong warfarin therapy
Answer the ff questions:
Given disease: Protein S deficiency
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) None
2) Autosomal dominant, autosomal recessive
3) a. Coagulation regulatory protein deficiency
b. Protein C will not have a cofactor during factor Va and VIIa inactivation
c. Heterozygous - decreased activity and concentration
d. Homozygous - will result to purpura fulminans
4) a. Associated w/ pregnancy, liver disease, renal disease, vitamin K deficiency, DIC
5) a. Skin necrosis
b. Thrombosis within the dermal vessels
c. Pruritus
6) a. Free protein S lvls: decreased
b. Total protein S lvls: decreased
c. Protein S activity assay: decreased
7) a. Lifelong warfarin therapy
Answer the ff questions:
Given disease: Activated Protein C Resistance
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) factor V Leiden mutation
2) Autosomal dominant
3) a. Coagulation factor defect
b. Mutation in the factor V gene makes it resistant to activated protein C
c. Factor Va will not be inactivated and will continuously initiate factor II activation
4) a. Described by Rogier Bertina in the city of Leiden, Netherlands
5) None
6) a. APC resistance assay: increased
b. FVL R506Q mutation: (+)
Answer the ff questions:
Given disease: Prothrombin Gene Mutation
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) prothrombin G20210A; prothrombin thrombophilia
2) Autosomal dominant
3) a. Coagulation factor defect
b. Consistent elevated prothrombin values
4) None
5) None
6) a. Factor II lvls: increased
b. Prothrombin G20210A mutation: (+)
7) None
Answer the ff questions:
Given disease: Systemic Fibrinolysis
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) Primary fibrinolysis
2) None
3) a. Fibrinolysis protein defect
b. Activation of plasminogen in the absence of a fibrin clot
4) a. Associated w/ late-phase cardiopulmonary bypass surgery, liver disease, transplant surgery, trauma, and amyloidosis
b. Lab results somehow mimic DIC except for the PLT ct
5) None
6) a. PT: prolonged
b. PTT: prolonged
c. TT: prolonged
d. Plasma fibrinogen: decreased
e. PLT ct: normal
f. D-dimer: (+)
7) None
Answer the ff questions:
Given disease: Antiphospholipid Syndrome
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) None
2) None
3) a. Antiphospholipid Abs
b. Abs act as non-sp inhibitors
c. Abs destroy protein-phospholipid complexes
4) a. Type of Ab is usually IgM
b. Alloimmune - associated w/ viral, fungal, bacterial or parasitic infection
c. Autoimmune - associated w/ SLE, RA, scleroderma, Sjogren syndrome
5) a. Transient ischemic attacks
b. Strokes
c. Recurrent pregnancy complications like stroke
6) a. PLT ct: decreased
b. PTT: prolonged
c. Lupus anticoagulant: detected
d. Anticardiolipin Ab: detected
e. Anti-beta(sign)2-glycoprotein 1 antibody: detected
f. Antiphosphatidylserine Ab: detected
7) None
Answer the ff questions:
Given disease: Arterial Thrombosis
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) None
2) None
3) a. Formation of atherosclerotic plaque
4) None
5) a. Peripheral vascular occlusion
b. Acute myocardial infarction
c. Cerebrovascular ischemia
6) a. Total cholesterol
b. LDL: increased
c. Lipoprotein: increased
d. CRP: increased
e. High-sensitivity CRP: increased
f. Plasma fibrinogen: increased
g. Plasma homocysteine: increased
h. Factor VIII: increased
7) a. AntiPLT drugs
b. Anti-inflammatory drugs
Answer the ff questions:
Given disease: Venous Thromboembolic Disease
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) Venous thrombosis
2) None
3) a. Formation of emboli
4) None
5) a. Deep venous thrombosis
b. Pulmonary embolism
6) a. D-dimer: (+)
7) None
Answer the ff questions:
Given disease: Disseminated Intravascular Coagulation
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) Defibrination syndrome; consumption coagulopathy
2) None
3) a. Combination of arterial and venous thrombosis
b. Simultaneous activation of PLTs, coagulation factors, and fibrinolytic proteins
4) a. Can be compensate or uncompensated
5) a. Systemic hemorrhage
b. Renal function impairment
c. Acute respiratory distress syndrome
d. Suden vision loss
e. Seizures
f. Skin, bone and bone marrow necrosis
g. Purpura and ecchymoses
6) a. PT: prolonged
b. PTT: prolonged
c. TT: prolonged
d. Plasma fibrinogen: decreased
e. PLT ct: decreased
f. Peripheral blood smear: presence of schistocytes
g. D-dimer: (+)
7) a. Treat underlying disorder
b. PLT concentrate
c. Plasma
What are the other conditions that results to thrombosis?
1) Liver disease
2) Vitamin K deficiency
Answer the ff questions:
Given disease: Liver Disease
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) None
2) None
3) a. Coagulation regulatory protein deficiency
b. Liver diseases suppress the biosynthetic function of hepatocytes
c. Coagulation regulatory proteins are not totally produced
4) a. Associated w/ hepatitis, cirrhosis, obstructive jaundice, cancer, poisoning, bilirubin metabolism disorders
5) None
6) None
7) None
Answer the ff questions:
Given disease: Vitamin K deficiency
1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?
1) None
2) None
3) a. Coagulation regulatory protein deficiency
b. Protein C, S, and Z are produced w/ abnormal forms, making them unfunctional and exhibit decreased activity
4) a. Associated w/ biliary duct obstruction, fat malabsorption, chronic diarrhea, and broad-spectrum antibiotics
5) None
6) None
7) a. Consumption of green leafy vegetables
b. Administration of vitamin K
