Heme 2.52 Flashcards Preview

hematology > Heme 2.52 > Flashcards

Flashcards in Heme 2.52 Deck (18)
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1
Q

what is hemophilia A

A

AKA factor VIII deficiency,

X-linked disorder; mutations in factor VIII gene

2
Q

what percentage of patieins with Hemophilia A have a family history of the disease

A

30%, disease is presumably caused by new mutations

3
Q

what is the prevalence of hemophilia A

A

affects about 1 in 5000 males

4
Q

what are the classifications of hemophilia A

A

% activity of VIII
5-25=mild
1-5=moderate
<0.5

5
Q

what is hemophilia B

A

X-linked disorder; Mutation in the factor IX gene

6
Q

what is the prevalence of hemophilia B

A

1 in 30000 males

7
Q

what are the risks associated with hemophila

A

increased bleeding with common injuries. in extreem cases, there can be spontaneous and without obvious causes of bleeding

8
Q

what are the serious places for bleeding to occure

A

joint capsules
skeletal muscles
GI tract
Brain

9
Q

what is the most serious symptom of hemophelia

A

joint bleeds (issues with the healing process)

10
Q

what is the most common inherited bleeding disorder

A

Von Willibrand’s disease(vWD)

11
Q

what are the two major roles of vWF

A

mediate platelet adhesion

spabiliing factor VIII

12
Q

what are the two majore causes of thrombocytopenia

A

A) impaired production of plateletes

B) increased destruction of platelets

13
Q

what can cause impaired producction of platelets

A

drugs

bone marrow failure

14
Q

what causes increased destruction of platelets

A

idiopathic thrombocytopenic perupura (ITP)

15
Q

what is thrombophilias

A

conditions associated with excessive clotting

16
Q

what are two example of inherited thrombophilias

A

protein C deficiencies

protein S deficiencies

17
Q

where are clots more most likely to form

A

in the veins

18
Q

activated protein C resistance

A

caused by a pour response of factor V to protein C