Hemorrhagic Flashcards

*Question: Which factor has the shortest plasma half-life and is the first to show decreased activity in liver disease?

A) Factor V

B) Factor X

C) Factor VII

D) Factor II

*Answer: C) Factor VII (6-hour half-life; PT is prolonged first)

*Question: What is the most prevalent inherited mucocutaneous bleeding disorder?

A) Hemophilia A

B) Von Willebrand Disease

C) Hemophilia B

D) Factor XIII deficiency

*Answer: B) Von Willebrand Disease

*Question: Which VWD subtype is the ONLY one with increased RIPA and decreased platelet count?

A) Type 1

B) Type 2A

C) Type 2B

D) Type 2N

*Answer: C) Type 2B

*Question: A patient has normal PT, prolonged APTT, and low FVIII:C with normal VWF:Ag. Which VWD subtype does this suggest?

A) Type 1

B) Type 2A

C) Type 2M

D) Type 2N

*Answer: D) Type 2N

*Question: Which laboratory test best distinguishes liver disease from Vitamin K deficiency?

A) Prothrombin time

B) Factor V assay

C) Thrombin time

D) APTT

*Answer: B) Factor V assay (low in liver disease; normal in Vitamin K deficiency)

*Question: What is the Trauma Triad of Death in Trauma-Induced Coagulopathy?

A) Hyperthermia, alkalosis, hypertension

B) Hypothermia, acidosis, hypoperfusion

C) Hypoxia, anemia, thrombocytopenia

D) Hypothermia, alkalosis, hypervolemia

*Answer: B) Hypothermia, acidosis, hypoperfusion

*Question: Which coagulation factors are Vitamin K-dependent?

A) FI, FII, FV, FVII

B) FII, FVII, FIX, FX

C) FVIII, FIX, FXI, FXII

D) FV, FVII, FX, FXIII

*Answer: B) FII, FVII, FIX, FX

*Question: A mixing study that does NOT correct after incubation indicates which pattern?

A) Factor deficiency

B) Inhibitor pattern

C) Fibrinogen deficiency

D) Platelet dysfunction

*Answer: B) Inhibitor pattern

*Question: Which assay is used to quantitate the auto-anti FVIII inhibitor level?

A) Bethesda Assay only

B) Mixing study

C) Nijmegen-Bethesda Assay

D) RIPA test

*Answer: C) Nijmegen-Bethesda Assay

*Question: In Hemophilia A, what is the typical PT and APTT result?

A) PT prolonged, APTT normal

B) PT normal, APTT prolonged

C) Both PT and APTT prolonged

D) Both PT and APTT normal

*Answer: B) PT normal, APTT prolonged

*Question: Where is VWF synthesized and stored in endothelial cells?

A) Alpha granules

B) Dense granules

C) Weibel-Palade bodies

D) Lysosomes

*Answer: C) Weibel-Palade bodies

*Question: Where is VWF stored in megakaryocytes/platelets?

A) Weibel-Palade bodies

B) Alpha granules

C) Dense granules

D) Beta granules

*Answer: B) Alpha granules

*Question: What is the chromosome location of the VWF gene?

A) Chromosome X

B) Chromosome 12

C) Chromosome 7

D) Chromosome 22

*Answer: B) Chromosome 12

*Question: What is the treatment for Trauma-Induced Coagulopathy?

A) Vitamin K supplementation

B) Desmopressin infusion

C) Massive Transfusion Protocol

D) Fresh frozen plasma only

*Answer: C) Massive Transfusion Protocol

*Question: A patient with liver disease has fibrinogen <100 mg/dL. What does this indicate?

A) Early mild liver disease

B) Vitamin K deficiency

C) End-stage liver disease

D) Dysfibrinogenemia only

*Answer: C) End-stage liver disease

*Question: Which factor is considered the specific marker for liver disease because it is non-Vitamin K dependent?

A) Factor VII

B) Factor X

C) Factor II

D) Factor V

*Answer: D) Factor V

*Question: In Chronic Renal Failure, what are the expected PT and APTT results?

A) Both prolonged

B) PT prolonged only

C) Both normal

D) APTT prolonged only

*Answer: C) Both normal

*Question: Which VWD subtype has absent large AND intermediate multimers?

A) Type 1

B) Type 2A

C) Type 2B

D) Type 2M

*Answer: B) Type 2A

*Question: Which immunoglobulin class is the most common inhibitor in Acquired Hemophilia A?

A) IgG1

B) IgG2

C) IgG3

D) IgG4

*Answer: D) IgG4

*Question: Hemophilia B is also known as:

A) Classical Hemophilia

B) Rosenthal Syndrome

C) Christmas Disease

D) Von Willebrand Disease

*Answer: C) Christmas Disease

*Question: Which factor is deficient in Hemophilia C (Rosenthal Syndrome)?

A) Factor VIII

B) Factor IX

C) Factor XI

D) Factor XII

*Answer: C) Factor XI

*Question: What is the genetic inheritance pattern of Hemophilia C?

A) X-linked recessive

B) Autosomal dominant

C) X-linked dominant

D) Autosomal recessive

*Answer: D) Autosomal recessive

*Question: A patient has PT prolonged and APTT normal. Which factor deficiency is most likely?

A) Factor VIII

B) Factor VII

C) Factor IX

D) Factor XI

*Answer: B) Factor VII

*Question: A patient has both PT and APTT normal but still has significant bleeding. Which deficiency should be suspected?

A) Factor VII deficiency

B) Factor VIII deficiency

C) Factor XIII deficiency

D) Factor X deficiency

*Answer: C) Factor XIII deficiency

*Question: What is the molecular weight range of VWF?

A) 50,000-500,000 Daltons

B) 500,000-20,000,000 Daltons

C) 100,000-1,000,000 Daltons

D) 285,000-5,000,000 Daltons

*Answer: B) 500,000-20,000,000 Daltons

*Question: ADAMTS13 functions by:

A) Activating fibrinogen to fibrin

B) Cleaving ultra-large VWF multimers

C) Inhibiting thrombin production

D) Activating Protein C

*Answer: B) Cleaving ultra-large VWF multimers

*Question: Decreased ADAMTS13 leads to which condition?

A) Hemophilia A

B) Von Willebrand Disease Type 3

C) Thrombotic Microangiopathy

D) Afibrinogenemia

*Answer: C) Thrombotic Microangiopathy

*Question: Which factors are lost in the urine in Nephrotic Syndrome?

A) FV, FVII, FX, FXIII

B) FII, FIX, FX, FXII

C) FVIII, FIX, FXI, FXII

D) FI, FII, FV, FVII

*Answer: B) FII, FIX, FX, FXII

*Question: Fibrinogen is elevated in which stage of liver disease?

A) Advanced liver disease

B) End-stage liver disease

C) Early/mild liver disease

D) Alcoholic cirrhosis only

*Answer: C) Early/mild liver disease

*Question: Which lab findings indicate dysfibrinogenemia?

A) Prolonged PT only

B) Prolonged APTT only

C) Both TT and reptilase time prolonged

D) Prolonged bleeding time only

*Answer: C) Both TT and reptilase time prolonged

*Question: What is the primary approach for treating VWD Type 2B?

A) Desmopressin

B) FVIII/VWF concentrate

C) Estrogen

D) Platelet transfusion

*Answer: B) FVIII/VWF concentrate

*Question: VWF protects FVIII from proteolysis and extends its half-life from:

A) 1-2 hours (free) to 4-6 hours (bound)

B) 2-4 hours (free) to 8-12 hours (bound)

C) 6-8 hours (free) to 12-24 hours (bound)

D) 30 minutes (free) to 2-4 hours (bound)

*Answer: B) 2-4 hours (free) to 8-12 hours (bound)

*Question: Which VWD subtype involves a mutation in the D-D3 domain causing reduced FVIII binding?

A) Type 2A

B) Type 2B

C) Type 2M

D) Type 2N

*Answer: D) Type 2N

*Question: Which of the following is NOT a Vitamin K-dependent factor?

A) Factor II

B) Factor V

C) Factor VII

D) Factor IX

*Answer: B) Factor V

*Question: What is the target FVIII level in Hemophilia A treatment?

A) 30-50 IU/dL

B) 50-70 IU/dL

C) 80-100 IU/dL

D) 100-120 IU/dL

*Answer: C) 80-100 IU/dL

*Question: Which condition causes Hemorrhagic Disease of the Newborn?

A) Congenital FVIII deficiency

B) Low FII, FVII, FIX, FX due to Vitamin K deficiency in newborns

C) Maternal thrombocytopenia

D) Congenital afibrinogenemia

*Answer: B) Low FII, FVII, FIX, FX due to Vitamin K deficiency in newborns

*Question: Which of the following can cause Acquired VWD?

A) Hemophilia A

B) Hypothyroidism

C) Vitamin K deficiency

D) Liver cirrhosis

*Answer: B) Hypothyroidism

*Question: In Hemophilia A, which is the most common inhibitor type and how does it react?

A) IgG1; cold-reacting

B) IgG4; non-complement fixing, warm-reacting

C) IgM; complement fixing

D) IgG2; heat-sensitive

*Answer: B) IgG4; non-complement fixing, warm-reacting

*Question: A patient with Acquired Hemophilia A has an inhibitor level >5 NBU. What is the treatment?

A) Large doses of FVIII

B) Platelet transfusion

C) Emicizumab or FEIBA

D) Fresh frozen plasma

*Answer: C) Emicizumab or FEIBA

*Question: What does the VWF:RCo assay measure?

A) VWF antigen concentration

B) Ristocetin cofactor activity measuring VWF's ability to agglutinate reagent platelets

C) Factor VIII coagulant activity

D) Collagen binding of VWF

*Answer: B) Ristocetin cofactor activity measuring VWF's ability to agglutinate reagent platelets

*Question: Which domain of VWF provides the carrier site for FVIII?

A) Domain A

B) Domain B

C) Domain C

D) Domain D

*Answer: D) Domain D

*Question: In VWD Type 3, what is the expected Factor VIII:C level?

A) Slightly low

B) Normal

C) Low (<10 units/dL)

D) Elevated

*Answer: C) Low (<10 units/dL)

*Question: What is the VWF:Ac/VWF:Ag ratio in VWD Type 1?

A) ≤0.7

B) >0.7

C) >1.0

D) N/A

*Answer: B) >0.7

*Question: A patient has low VWF:Ac, normal to slightly decreased VWF:Ag, ratio ≤0.7, and large/intermediate multimers absent. Which VWD subtype is this?

A) Type 1

B) Type 2A

C) Type 2B

D) Type 2M

*Answer: B) Type 2A

*Question: Which of the following is considered the largest molecule in human plasma?

A) Fibrinogen

B) Factor VIII

C) Von Willebrand Factor

D) Immunoglobulin M

*Answer: C) Von Willebrand Factor

*Question: Hemophilia A is also known as:

A) Christmas Disease

B) Rosenthal Syndrome

C) Classical Hemophilia

D) Von Willebrand Disease

*Answer: C) Classical Hemophilia

*Question: Factor VIII is translated from which chromosome?

A) Chromosome 12

B) Chromosome Y

C) Chromosome X

D) Chromosome 7

*Answer: C) Chromosome X

*Question: In Hemophilia A genetics, which individuals experience anatomic bleeding?

A) Female heterozygotes

B) Male hemizygotes

C) Female homozygotes

D) Both male and female equally

*Answer: B) Male hemizygotes

*Question: Which of the following best describes localized hemorrhage?

A) Bleeding from multiple sites simultaneously

B) Spontaneous and recurring bleeds

C) Bleeding at a single location due to injury, infection, or tumor

D) Bleeding associated with thrombocytopenia

*Answer: C) Bleeding at a single location due to injury, infection, or tumor

*Question: What is the plasma concentration of VWF?

A) 0.1-0.3 mg/dL

B) 0.5-1.0 mg/dL

C) 1.5-2.0 mg/dL

D) 2.5-5.0 mg/dL

*Answer: B) 0.5-1.0 mg/dL

*Question: Petechiae are defined as skin lesions with a diameter of:

A) >3 cm

B) 3 mm to 1 cm

C) >1 cm

D) <3 mm

*Answer: D) <3 mm

*Question: Mucocutaneous hemorrhage is associated with which platelet count threshold?

A) <100,000/µL

B) <50,000/µL

C) <20,000/µL

D) <5,000/µL

*Answer: C) <20,000/µL

*Question: Which test is the most reproducible VWF assay and used to monitor treatment?

A) VWF:RCo

B) VWF:Ag

C) RIPA

D) VWF:CB

*Answer: B) VWF:Ag

*Question: In liver disease, which proteins are produced in the des-γ-carboxyl form?

A) Fibrinogen and albumin

B) Vitamin K-dependent factors and control proteins

C) VWF and FXIII

D) FVIII and FV

*Answer: B) Vitamin K-dependent factors and control proteins

*Question: What is the primary treatment for mild to moderate Hemophilia A?

A) Human plasma-derived FVIII + antifibrinolytic

B) Desmopressin + antifibrinolytic agent

C) VWF concentrate

D) Platelet transfusion

*Answer: B) Desmopressin + antifibrinolytic agent

*Question: Which condition is characterized by fibrin deposition in renal vessels leading to renal failure?

A) Nephrotic syndrome

B) Hemostasis Activation Syndrome (DIC, HUS, TTP)

C) Chronic Renal Failure with Vitamin K deficiency

D) Acquired VWD

*Answer: B) Hemostasis Activation Syndrome (DIC, HUS, TTP)

*Question: Ecchymoses are defined as skin lesions with a diameter of:

A) <3 mm

B) 3 mm to 1 cm

C) >1 cm

D) <1 mm

*Answer: C) >1 cm

*Question: Which control proteins are Vitamin K-dependent?

A) Protein A, B, C

B) Protein C, S, Z

C) Protein X, Y, Z

D) Protein S, T, U

*Answer: B) Protein C, S, Z

*Question: FXIII deficiency results in which PT and APTT findings?

A) PT prolonged, APTT normal

B) PT normal, APTT prolonged

C) Both prolonged

D) Both normal

*Answer: D) Both normal

*Question: What follow-up test is indicated when both PT and APTT are normal but FXIII deficiency is suspected?

A) Fibrinogen assay

B) Mixing study

C) FXIII quantitative assay

D) Bethesda assay

*Answer: C) FXIII quantitative assay

*Question: Which VWD Type has ALL multimers absent on multimer analysis?

A) Type 2A

B) Type 2B

C) Type 2M

D) Type 3

*Answer: D) Type 3

*Question: In VWD Type 2M, what is the underlying defect?

A) Increased GP1b binding

B) Reduced FVIII binding

C) Defective collagen or GP1b binding with normal multimers

D) Impaired VWF secretion

*Answer: C) Defective collagen or GP1b binding with normal multimers

*Question: What is the primary treatment for VWD Type 3?

A) Desmopressin

B) Estrogen

C) VWF concentrate

D) Fresh frozen plasma

*Answer: C) VWF concentrate

*Question: Hemophilia B involves deficiency of which factor?

A) Factor VIII

B) Factor IX

C) Factor XI

D) Factor XII

*Answer: B) Factor IX

*Question: Factor IX in Hemophilia B is best described as:

A) Non-Vitamin K dependent serine protease

B) Vitamin K-dependent serine protease

C) Vitamin K-dependent carboxylase

D) Acute phase reactant

*Answer: B) Vitamin K-dependent serine protease

*Question: Which lab finding is expected in Hemophilia B?

A) PT prolonged, APTT normal, TT normal

B) PT normal, APTT prolonged, TT and fibrinogen normal

C) Both PT and APTT prolonged

D) PT normal, APTT normal, FXIII low

*Answer: B) PT normal, APTT prolonged, TT and fibrinogen normal

*Question: Which of the following is an acute phase reactant in liver disease that may be unaffected or elevated?

A) Factor V

B) Factor X

C) VWF, Factor VII, Factor XIII

D) Factor II

*Answer: C) VWF, Factor VII, Factor XIII

*Question: In systemic shock during TIC, release of inflammatory cytokines leads to consumption of which enzyme?

A) Protein C

B) ADAMTS13

C) Antithrombin III

D) Plasminogen activator

*Answer: B) ADAMTS13

*Question: Which of the following is the VWF:Ac/VWF:Ag ratio in VWD subtypes 2A, 2B, and 2M?

A) >0.7

B) ≤0.7

C) >1.0

D) N/A

*Answer: B) ≤0.7

*Question: What is the incidence of Hemophilia C in the general population?

A) 1 in 10,000

B) 1 in 100,000

C) 1 in 1,000,000

D) 1 in 500,000

*Answer: C) 1 in 1,000,000

*Question: VWF gene consists of how many exons spanning how many kilobases?

A) 26 exons, 100 kb

B) 52 exons, 178 kb

C) 78 exons, 250 kb

D) 40 exons, 150 kb

*Answer: B) 52 exons, 178 kb

*Question: Which of the following is true regarding Factor V in liver disease vs. Vitamin K deficiency?

A) FV is low in both conditions

B) FV is normal in liver disease but low in Vitamin K deficiency

C) FV is low in liver disease but normal in Vitamin K deficiency

D) FV is elevated in both conditions

*Answer: C) FV is low in liver disease but normal in Vitamin K deficiency

*Question: What is the formula for calculating plasma volume in Hemophilia A treatment?

A) Weight (kg) × 70 mL/kg × hematocrit

B) Weight (kg) × 65 mL/kg × (1 − hematocrit)

C) Weight (kg) × 60 mL/kg × (1 + hematocrit)

D) Weight (kg) × 55 mL/kg × (1 − hematocrit)

*Answer: B) Weight (kg) × 65 mL/kg × (1 − hematocrit)

*Question: Which of the following congenital bleeding disorders presents with both mucocutaneous AND anatomic bleeding when VWF is <30 IU/dL?

A) Hemophilia A

B) Factor XIII deficiency

C) Von Willebrand Disease

D) Factor VII deficiency

*Answer: C) Von Willebrand Disease

*Question: In VWD, which range of VWF level is classified as "Low VWF" individuals who experience bleeding?

A) 10-20 IU/dL

B) 30-50 IU/dL

C) 50-70 IU/dL

D) 20-30 IU/dL

*Answer: B) 30-50 IU/dL

*Question: Which assay is used to confirm VWD Type 2N?

A) RIPA

B) VWF:CB

C) VWF:FVIIIB assay or genetic testing

D) VWF:RCo

*Answer: C) VWF:FVIIIB assay or genetic testing

*Question: Which of the following is the most sensitive early marker of liver disease coagulopathy?

A) APTT prolongation

B) Decreased platelet count

C) PT prolongation

D) Low fibrinogen

*Answer: C) PT prolongation

*Question: What type of bleeding does Hemophilia A primarily cause?

A) Mucocutaneous bleeding

B) Anatomic bleeding (deep muscle and joint hemorrhage)

C) Localized bleeding only

D) Gastrointestinal bleeding only

*Answer: B) Anatomic bleeding (deep muscle and joint hemorrhage)

*Question: Recurrent bleeding in Hemophilia A joints is called:

A) Hematuria

B) Hemarthroses

C) Petechiae

D) Purpura

*Answer: B) Hemarthroses

*Question: Which of the following causes Acquired Hemophilia A to be triggered in pregnant women?

A) AHA Type 1

B) AHA Type 2

C) AHA Type 3

D) AHA Type 4

*Answer: B) AHA Type 2

*Question: In Vitamin K deficiency, which PT and APTT pattern is expected?

A) PT normal, APTT prolonged

B) PT prolonged with or without prolonged APTT

C) Both normal

D) APTT prolonged only

*Answer: B) PT prolonged with or without prolonged APTT

*Question: Which of the following is the VWF:Ac/VWF:Ag ratio in VWD Type 1?

A) ≤0.7

B) N/A

C) >0.7

D) >1.0

*Answer: C) >0.7

*Question: Vitamin K is best described as:

A) Water-soluble and absorbed without bile salts

B) Fat-soluble and requires bile salts for absorption

C) Water-soluble and requires active transport

D) Fat-soluble and absorbed independently of bile

*Answer: B) Fat-soluble and requires bile salts for absorption

*Question: Which domain of VWF supports binding sites for collagen and platelet GP 1b/IX/V?

A) Domain B

B) Domain C

C) Domain D

D) Domain A

*Answer: D) Domain A

*Question: Which domain of VWF provides a site that binds platelet receptor GPIIb/IIIa?

A) Domain A

B) Domain B

C) Domain C

D) Domain D

*Answer: C) Domain C

*Question: In Factor XIII deficiency, which clinical feature is characteristic besides bleeding?

A) Petechiae

B) Epistaxis

C) Poor wound healing

D) Hemarthroses

*Answer: C) Poor wound healing

*Question: What is the RIPA result in VWD Type 2B?

A) Decreased

B) Normal

C) Absent

D) Increased

*Answer: D) Increased

*Question: Which of the following autoimmune conditions is associated with Acquired VWD?

A) Rheumatoid arthritis

B) Lupus (SLE)

C) Grave's disease

D) Sjögren's syndrome

*Answer: B) Lupus (SLE)

*Question: In afibrinogenemia, what is the recommended treatment goal?

A) Raise fibrinogen to >100 mg/dL using FFP

B) Raise fibrinogen to >150 mg/dL using fibrinogen concentrate or CRYO

C) Raise fibrinogen to >200 mg/dL using PCC

D) No treatment required

*Answer: B) Raise fibrinogen to >150 mg/dL using fibrinogen concentrate or CRYO

*Question: Which factor deficiency results in moderate to severe anatomic bleeding and is treated with rFVIIa or PCC?

A) Factor V deficiency

B) Factor XI deficiency

C) Factor VII deficiency

D) Factor XIII deficiency

*Answer: C) Factor VII deficiency

*Question: In the mixing study for Hemophilia A inhibitor, the 1:1 mix ratio means:

A) 1 part patient + 1 part saline

B) 1 part patient + 1 part Normal Pooled Plasma

C) 4 parts patient + 1 part Normal Pooled Plasma

D) 1 part patient + 4 parts Normal Pooled Plasma

*Answer: B) 1 part patient + 1 part Normal Pooled Plasma

*Question: Which of the following is used to confirm VWD Type 2B specifically?

A) VWF:Ag test

B) RIPA test positive

C) Genetic testing only

D) Factor V assay

*Answer: B) RIPA test positive

*Question: What is the expected APTT result in VWD Type 3?

A) Normal

B) Slightly decreased

C) Prolonged

D) Cannot be determined

*Answer: C) Prolonged

*Question: A patient with liver disease is found to have VWF levels that are elevated. This is because VWF is produced by:

A) Hepatocytes

B) Endothelial cells and megakaryocytes, not the liver

C) The spleen

D) Bone marrow stromal cells only

*Answer: B) Endothelial cells and megakaryocytes, not the liver

*Question: In Hemophilia A with inhibitors, a low responder is defined as an inhibitor level of:

A) ≤2 NBU

B) ≤5 NBU

C) ≤10 NBU

D) ≤15 NBU

*Answer: B) ≤5 NBU

*Question: Which type of bleeding is associated with coagulopathies such as Hemophilia, involving joints and deep tissues?

A) Mucocutaneous hemorrhage

B) Anatomic hemorrhage

C) Localized hemorrhage

D) Petechial hemorrhage

*Answer: B) Anatomic hemorrhage