Question 1

Which of the following is used in the identification of a secondary hemostatic pathway anomaly?&#10;A)Bleeding time&#10;B)ACE activity&#10;C)Platelet aggregation studies&#10;D)Factor assays

Accepted Answer

Factor assays are used to identify a secondary hemostatic pathway anomaly as they help determine the levels of specific clotting factors in the blood. Bleeding time is used to evaluate primary hemostasis, ACE activity is a test for hypertension or heart failure, and platelet aggregation studies are also used to evaluate primary hemostasis.

Question 2

Interpret the following results: PT: 11.8 sec (RR: 11.2-13.8 sec)
INR: 1.07
APTT: 57 sec (RR: 23-32 sec)
BT: 12 min
Ristocetin aggregation: decreased aggregation
Based on this information, from what is the patient most likely suffering?

A)Hemophilia A
B)Bernard-Soulier syndrome
C)DIC
D)von Willebrand's disease

von Willebrand's disease

Accepted Answer

The PT and INR are within normal limits, so it is unlikely that the patient has a coagulation factor deficiency such as Hemophilia A or Bernard-Soulier syndrome. The APTT is prolonged, indicating a possible deficiency in the intrinsic pathway factors, which can be seen in von Willebrand's disease. The BT is prolonged, which can also be seen in von Willebrand's disease due to defective platelet function. The decreased Ristocetin aggregation also supports a diagnosis of von Willebrand's disease.

Question 3

A 5-year-old boy has his blood drawn for platelet aggregation studies.Ristocetin aggregation (RIPA) comes back abnormal with all other agonists displaying normal aggregation.VWF antigen is within normal limits.What is the most likely reason for this discrepancy?

A)The VWF antigen test was performed incorrectly, and should be repeated.
B)The patient might be suffering from a qualitative platelet defect disorder.
C)The RIPA exam was performed incorrectly, and should be repeated.
D)Multimer analysis should be performed to confirm these findings.

Accepted Answer

Abnormal RIPA with normal VWF antigen suggests a qualitative platelet defect disorder, also known as Bernard-Soulier syndrome or Glanzmann thrombasthenia. Multimer analysis is not necessary in this scenario. The VWF antigen test is likely accurate as it was within normal limits. The RIPA exam should be repeated to confirm the abnormal result.

Question 4

What disorder is the patient most likely suffering from?&#10;A)von Willebrand's disease&#10;B)DIC&#10;C)A possible hemophilia&#10;D)Lupus anticoagulant

Accepted Answer

The patient is presenting with hematomas after a minor injury and prolonged APTT, which could indicate a possible hemophilia. Platelet aggregation studies are normal, which rules out von Willebrand's disease. DIC typically presents with increased coagulation, not prolonged APTT. Lupus anticoagulant is a possibility, but the clinical presentation suggests hemophilia as the more likely diagnosis. The best choice would be to perform factor VIII and IX assays to confirm or rule out hemophilia.

Question 5

Questions 5-7: Use this case to answer the following questions: ?A 5-year-old boy visits his primary care physician after an accident on his bike.It was his first accident, and he fell on his knees.His mother noticed severe swelling in his knees and elbows, but thought that it was from the accident.She placed an ice pack on the affected areas and put him to rest.After a few days, the swelling had not improved, so she made an appointment to see his pediatrician.The physician asks him a few questions about the accident and then proceeds to perform a physical examination.The physician notes small hematomas on his elbows and knees and also on his upper torso.He draws blood, and the results are shown below. PT: 12.2 sec (RR: 11.3-12.8 sec)
INR: 1.12
APTT: 72 sec (RR: 23-32 sec)
Platelet aggregation studies: normal aggregation with all agonists
The physician admits him to the area hospital.What should be his next course of action in investigating these results?

A)He should repeat the aggregation studies.
B)He should start the patient on Coumadin.
C)He should order factor assays of the intrinsic pathway.
D)He should order factor assays of the intrinsic, extrinsic, and common pathways.

Accepted Answer

The prolongation of APTT suggests a possible deficiency in the intrinsic pathway factors (factors VIII, IX, XI, and XII). Thus, further testing should be done to confirm this suspicion. Starting the patient on Coumadin, which inhibits vitamin K-dependent clotting factors in the extrinsic pathway, would not be appropriate without further investigation. Repeat aggregation studies would not yield any additional information. Factor assays for the extrinsic and common pathways would not be necessary unless there were other indications for a coagulation disorder.

Question 6

Which of the following clinical symptoms correlates with a severe form of hemophilia A or B?&#10;A)Bleeding at circumcision with a factor level of 1-5 U/dL&#10;B)Excessive bleeding after surgery or trauma with factor levels of 6-30 U/dL&#10;C)Frequent spontaneous hemarthroses&#10;D)Infrequent spontaneous joint and tissue bleeds

Accepted Answer

Frequent spontaneous hemarthroses (joint bleeds) are a hallmark symptom of severe hemophilia A or B. Bleeding at circumcision with a factor level of 1-5 U/dL is a common presentation of moderate hemophilia, while excessive bleeding after surgery or trauma with factor levels of 6-30 U/dL is a common presentation of mild to moderate hemophilia. Infrequent spontaneous joint and tissue bleeds are a common presentation of mild hemophilia.

Question 7

A physician has confirmed von Willebrand's disease in his patient.Further testing is needed to determine which subtype the patient has.Multimer analysis is performed, and comes back normal.What is the most probable explanation for this?

A)The patient is not suffering from von Willebrand's disease.
B)The patient is suffering from type I VWD.
C)The patient has BSS.
D)The wrong patient was drawn.

Accepted Answer

Type I VWD is characterized by a quantitative decrease in von Willebrand factor (VWF), but the multimers are usually normal. Therefore, a normal multimer test in the presence of VWF deficiency is consistent with type I VWD. Option A is incorrect because the patient has already been confirmed to have VWD. Option C is incorrect because Bernard-Soulier syndrome (BSS) is characterized by a deficiency or dysfunction of glycoprotein Ib. Option D is unlikely and not a probable explanation.

Question 8

F-VIII deficiency and F-IX deficiency are classified as what category of disorders?&#10;A)Autosomal dominant&#10;B)X-linked recessive disorders&#10;C)Autosomal recessive&#10;D)X-linked dominant

Accepted Answer

F-VIII deficiency and F-IX deficiency are both caused by mutations in genes located on the X chromosome and are therefore X-linked recessive disorders. These disorders typically affect males more severely than females, as females have two X chromosomes (and therefore a backup copy of the gene) while males only have one X chromosome.

Question 9

What term describes hereditary or acquired disorders and relates to the absence of a coagulation protein, or to a protein that is present in the plasma but that is functionally defective?&#10;A)Circulating anticoagulant&#10;B)Lupus anticoagulant&#10;C)Deficiency&#10;D)Dysfunctional

Accepted Answer

The term "deficiency" specifically refers to the absence of a coagulation protein or a functionally defective protein in the context of hereditary or acquired disorders related to coagulation. Choice A (circulating anticoagulant) and choice B (Lupus anticoagulant) both refer to specific types of antibodies that can affect coagulation but are not necessarily related to a deficiency in coagulation proteins. Choice D (dysfunctional) is a general term that could apply to any protein or system in the body and does not specifically relate to coagulation disorders.

Question 10

Hemophilia A is caused by a deficiency in which of the following proteins?&#10;A)von Willebrand's factor&#10;B)Factor VIII&#10;C)Factor IX&#10;D)Glycoprotein IIb/IIIa

Accepted Answer

Hemophilia A is caused by a deficiency in Factor VIII, which is essential for blood clotting.

Question 11

A patient might be suffering from a coagulopathy.Which of the following physical manifestations would suggest a primary hemostatic pathway problem?&#10;A)Hematomas&#10;B)Petechiae&#10;C)Joint and muscle bleeding&#10;D)Ecchymoses

Accepted Answer

The answer of A patient might be suffering from a...

Question 12

A patient with a protein C deficiency is at risk for developing which of the following?&#10;A)Thrombosis&#10;B)Hemorrhage&#10;C)Lupus anticoagulant inhibitor&#10;D)Factor deficiency

Accepted Answer

The answer of A patient with a protein C deficiency...

Question 13

A patient has a prolonged PT.What can you infer from this result?&#10;A)The patient has a deficiency in the coagulation factors of the extrinsic pathway.&#10;B)The patient has a deficiency in the coagulation factors of the intrinsic pathway.&#10;C)The patient has a deficiency in platelets.&#10;D)The patient has a deficiency in the coagulation factors of the common pathway.

Accepted Answer

The answer of A patient has a prolonged PT.What can...

Question 14

Predict results that will confirm the patient's diagnosis.&#10;A)Abnormal aggregation with Ristocetin&#10;B)An increased D-dimer&#10;C)Decreased activity of either factors VIII or IX&#10;D)The presence of an inhibitor pattern in a mixing study

Accepted Answer

The answer of Predict results that will confirm the patient's...

Question 15

Screening tests for the evaluation of VWD include the platelet count, APTT, PT, the bleeding time, and/or the PFA-100.Which of the following results would be evident in most cases of VWD?&#10;A)Platelet count low&#10;B)Ristocetin platelet aggregation test decreased&#10;C)rRstocetin platelet aggregation test normal&#10;D)VWF: Ag assay normal

Accepted Answer

The answer of Screening tests for the evaluation of VWD...

Question 16

Which congenital deficiency(s) is suspected with a normal platelet count, an abnormal PT and APTT, and normal PFA-100 and thrombin time?&#10;A)Factor VII&#10;B)Factors X, V, II (prothrombin)&#10;C)Factor VIII&#10;D)Fibrinogen

Accepted Answer

The answer of Which congenital deficiency(s) is suspected with a...

Question 17

A 34-year-old woman has just given birth to a healthy baby boy.After the delivery of the baby, the physician proceeds to deliver the placenta.After it has been extracted from the mother's uterus, the resident examines it and notes that it appears incomplete.They try to deliver the remainder, but are unsuccessful.A few moments later, the mother loses consciousness, and is bleeding profusely from her uterus.Blood is collected, and the results are shown below. PT: 17 sec (RR: 11.3-12.8 sec)
INR: 3.02
APTT: 67 sec (RR: 23-32 sec)
D-dimer: 540 ng/mL (RR: <400 ng/mL)
Fibrinogen: 82 mg/dL (RR: 200-400 mg/dL)
Factor V assay: 12%
Based on this information, from what is the patient most likely suffering?

A)Hemophilia A
B)von Willebrand's disease
C)Placenta previa
D)DIC

Accepted Answer

The answer of A 34-year-old woman has just given birth...

Question 18

What is the inheritance pattern of hemophilia A?&#10;A)Autosomal dominant&#10;B)Autosomal recessive&#10;C)Sex-linked&#10;D)None of the above

Accepted Answer

The answer of What is the inheritance pattern of hemophilia...

Question 19

Deficiency of coagulation proteins is defined as:&#10;A)A decrease in the concentration of the coagulation proteins.&#10;B)A decrease in the concentration of the coagulation proteins and a subsequent decrease in the functionality of those proteins.&#10;C)A decrease in the concentration of the coagulation proteins or impaired functionality of those proteins.&#10;D)A decrease in platelets that causes a decreased functionality of the coagulation proteins.

Accepted Answer

The answer of Deficiency of coagulation proteins is defined as:&#10;A)A...

Question 20

A preterm infant (29 weeks old) has a platelet count of 85 x 109/L.Is this a normal finding? Why or why not?&#10;A)No, it is an abnormal finding, because infants have normal adult amounts of platelets after 27 weeks' gestation.&#10;B)Yes, this is a normal finding, because preterm infants have decreased platelet counts due to abnormal birthing circumstances.&#10;C)Yes, this is a normal finding, because platelet development is similar to lung development in an infant; levels are dependent upon the age of gestation.&#10;D)No, this is an abnormal finding, because preterm infants are subject to DIC, and that results in a decreased platelet count.

Accepted Answer

The answer of A preterm infant (29 weeks old) has...

Question 21

Explain how disseminated intravascular coagulation is not a primary disease state.Correlate etiology, pathophysiology, and laboratory analysis in your response.&#10;

Accepted Answer

The answer of Explain how disseminated intravascular coagulation is not...

Question 22

Explain the inheritance pattern of the following disorders, and explain how this contributes to the clinical manifestations of the disorders:&#10;A)Hemophilia A&#10;

Accepted Answer

The answer of Explain the inheritance pattern of the following...

Question 23

What plasma factor levels can be expected in a female carrier of F-VIII or F-IX deficiency?&#10;A)25% of the normal plasma levels of the deficient factor&#10;B)12.5% of the normal plasma levels of the deficient factor&#10;C)50% of the normal plasma levels of the deficient factor&#10;D)5% of the normal plasma levels of the deficient factor

Accepted Answer

The answer of What plasma factor levels can be expected...

Question 24

Which of the following laboratory assays would be useful in identifying presence of primary fibrinolysis?&#10;A)Fibrinogen and FDP&#10;B)FDP and D-dimer&#10;C)D-dimer and plasmin&#10;D)Fibrinogen and platelet count

Accepted Answer

The answer of Which of the following laboratory assays would...

Question 25

A known hemophiliac has developed an inhibitor.The physician in charge of his care wants to know how much inhibitor is present so that he can properly treat him.What two lab tests would he have to order to assess this?

A)APTT; factor assay
B)Factor VIII: C level; Bethesda titer
C)VWF antigen; Bethesda titer
D)Type and screen; factor assay

Accepted Answer

The answer of A known hemophiliac has developed an inhibitor.The...

Question 26

What test(s) would help differentiate this disorder from other acquired disorders?&#10;A)D-dimer&#10;B)Liver enzymes&#10;C)von Willebrand's factor&#10;D)All of the above

Accepted Answer

The answer of What test(s) would help differentiate this disorder...

Question 27

Explain how liver disease can be differentiated from DIC through laboratory test analysis.

Accepted Answer

The answer of Explain how liver disease can be differentiated...

Question 28

An advantage of prenatal diagnosis by genotypic analysis for hemophilia over phenotypic analysis is:&#10;A)ABO blood type does not affect the analysis.&#10;B)A chorionic villus biopsy can be tested at 4 weeks gestation.&#10;C)Direct DNA diagnosis is available for all familes.&#10;D)It can be done as a PC test.

Accepted Answer

The answer of An advantage of prenatal diagnosis by genotypic...

Question 29

How does von Willebrand's disease differ from Bernard-Soulier syndrome? Correlate the pathophysiology of both disorders in your response.Name at least two laboratory test methods by which to differentiate between the two.

Accepted Answer

The answer of How does von Willebrand's disease differ from...

Question 30

Which is the most common F-VIII mutation in patients with a severe phenotype (occurring in almost 50% of patients)?&#10;A)Gross deletion of entire gene locus&#10;B)Point mutation involving the thrombin cleavage site&#10;C)Inversion mutation of intron 22&#10;D)Point mutation involving the site of VWF attachment

Accepted Answer

The answer of Which is the most common F-VIII mutation...

Question 31

George Jones is admitted to the hospital with abdominal pain.Upon physical examination, the attending physician notices a yellow ring around George's green eyes.George has a significant swelling in his abdomen, and has been suffering from swollen joints for the last month.The physician runs a battery of labs.The results are below: $\begin{array}{|l|l|l|}\hline\text { TEST } & \text { George Jones } & \text { Reference Range } \\\hline \text { PT } & 16.8 \mathrm{sec} & 11-13 \mathrm{sec} \\\hline \text { INR } & 3.85 & \text { NA } \\\hline \text { APTT } & 71 \mathrm{sec} & 23-32 \mathrm{sec} \\\hline \text { BT } & 5 \mathrm{~min} & 2-6 \mathrm{~min} \\\hline \text { Fibrinogen } & 275 \mathrm{mg} / \mathrm{dL} & 200-400 \mathrm{mg} / \mathrm{dL} \\\hline \text { Factor X } & 22 \% & >50 \% \text { activity } \\\hline \text { Factor II } & 21 \% & >50 \% \text { activity } \\\hline \text { Factor VII } & 23 \% & >50 \% \text { activity } \\\hline \text { Factor V } & 28 \% & >50 \% \text { activity } \\\hline \text { Factor XII } & 95 \% & >50 \% \text { activity } \\\hline \text { Factor XI } & 99 \% & >50 \% \text { activity } \\\hline \text { Factor IX } & 30 \% & >50 \% \text { activity } \\\hline \text { RIPA } & 95 \% & >50 \% \text { activity } \\\hline \text { AST } & \text { Elevated } & \text { Normal } \\\hline \text { ALT } & \text { Elevated } & \text { Nomal } \\\hline \text { GGT } & \text { Elevated } & \text { Normal } \\\hline \text { TBL } & \text { Elevated } & \text { Normal } \\\hline \text { DBI } & \text { Elevated } & \text { Nomal } \\\hline\end{array}$ Based on this information, from what is George most likely suffering?

A) Liver disease
B) Vitamin K deficiency
C) Disseminated intravascular coagulation
D) Massive cerebral hemorrhage

Accepted Answer

The answer of George Jones is admitted to the hospital...

Question 32

What tests typically are utilized in the detection of lupus anticoagulants in the laboratory?

Accepted Answer

The answer of What tests typically are utilized in the...

Question 33

What type of VWD is the only type to have a decreased platelet count?&#10;A)Type 1&#10;B)Type 3&#10;C)Type 2B&#10;D)Type 2N

Accepted Answer

The answer of What type of VWD is the only...

Question 34

Which assay would assess for the presence of a circulating nonspecific inhibitor?&#10;A)Bethesda titer&#10;B)Factor-specific inhibitor&#10;C)Lupus anticoagulant&#10;D)D-dimer

Accepted Answer

The answer of Which assay would assess for the presence...

Question 35

Deficiencies of the fibrin-forming proteins often have a delayed bleeding symptom that is due to which of the following?&#10;A)Excessive bleeding from a traumatic injury&#10;B)Bleeding from rupture of small arterioles&#10;C)Formation of hematomas&#10;D)Absence of hemostatic plug stabilization with fibrin.

Accepted Answer

The answer of Deficiencies of the fibrin-forming proteins often have...

Deck 31: Disorders of Secondary Hemostasis