Deck 10: Hemophilia

Question 1

Hemophilias are generally inherited in an X-linked pattern. How will this affect the offspring of a father with a normal gene and a carrier mother?

A) All sons will be affected and 50% of daughters will be carriers
B) No children will be affected but all will be carriers.
C) A 25% chance of having a son who is affected and a 25% chance of having a daughter who is a carrier, and 50% chance that a child (either boy or girl) does not have the gene at all.
D) 50% of sons are affected and all daughters are carriers.

Answer 1

C
Explanation: This couple has a 25% chance of having a son who is affected and a 25% chance of having a daughter who is a carrier, and 50% chance that a child (either boy or girl) does not have the gene at all.

Question 2

Hemophilias are generally inherited in an X-linked pattern. How will this affect the offspring of an affected father and normal gene mother?

A) All sons will be affected and 50% of daughters will be carriers
B) No children will be affected but all will be carriers.
C) A 25% chance of having a son who is affected and a 25% chance of having a daughter who is a carrier, and 50% chance that a child (either boy or girl) does not have the gene at all.
D) All sons will be normal and all daughters are carriers.

Answer 2

D
Explanation: In the case of this couple, all sons will be normal and all daughters are carriers.

Question 3

A 27-year-old man with a history of bleeding while brushing and flossing his teeth presents with a nosebleed. This episode is worse than ones he has experienced in the past. You suspect he may have this coagulopathy, the most common inherited bleeding disorder in the United States.

A) Von Willebrand disease
B) Hemophilia A
C) Hemophilia B
D) Hemophilia B Leyden

Answer 3

A
Explanation: Von Willebrand Disease is the most common inherited bleeding disorder in the United States.

Question 4

A 27-year-old man with a history of bleeding while brushing and flossing his teeth presents with a nosebleed. This episode is worse than ones he has experienced in the past. Vital signs are stable, however, applying pressure has failed to control bleeding. What the next step in treatment?

A) Platelets
B) Desmopressin
C) Cryoprecipitate
D) Fresh Frozen Plasma

Question 5

A 12-year-old boy with a history of bleeding while flossing his teeth presents with a nosebleed. He has had recurrent nosebleeds over the past 5 years. His parents deny a family history of bleeding disorders. You suspect von Willebrand disease. A decreased vWF along with what other lab findings confirms the diagnosis?

A) Normal PT & bleeding time, prolonged aPTT
B) Normal aPTT & bleeding time, prolonged PT
C) Prolonged PT & aPTT, normal bleeding time
D) Normal PT & aPTT and prolonged bleeding time

Question 6

A 43-year-old woman who has von Willebrand disease presents with a nosebleed that she can't control. She is currently menstruating and using intranasal DDAVP. Vital signs reveal tachycardia and a BP of 98/68. What treatment is indicated at this time?

A) Platelets
B) vWF Factor containing Factor VIII concentrate
C) Fresh Frozen Plasma
D) IV Desmopressin

Question 7

What is the first line treatment for a patient who has Hemophilia B who presents with minor bleeding?

A) Platelets
B) Cryoprecipitate
C) IX concentrate
D) Desmopressin

Question 8

The following pair matches coagulation factors with the correct pathway in the coagulation cascade:

A) Factor VIII & IX- extrinsic
B) Factor VIII & IX - intrinsic
C) Factor VIII & IX - common
D) Factor VII & IX - intrinsic

Question 9

This bleeding disease is most frequently inherited in an autosomal dominant pattern:

A) Hemophilia A
B) Von Willebrand disease
C) Hemophilia B
D) Hemophilia B Leyden

Question 10

A patient who has a deficiency in Factor VIII has what disease?

A) Hemophilia A
B) Hemophilia B
C) Hemophilia B Leyden
D) Von Willebrand disease