INHERITED DISORDERS OF COAGULATION Flashcards

1
Q

Hemophilias is AKA?

A

Love hemorrhage

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2
Q

Hemophilias are characterized by a deficiency of the?

A

Antihemophilic factors

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3
Q

Enumerate the inherited disorders of coagulation

A

Hemophilia A
Hemophilia B
Hemophilia C
Prekallikrein; HMWK; FACTOR XII DEFICIENCY

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4
Q

This is the most common type of hemophilia

A

Hemophilia A

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5
Q

Hemophilia A is AKA?

A

Classic hemophilia

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6
Q

This is a sex linked disorder characterized by a deficiency in factor VIII:C

A

Hemophilia A

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7
Q

Hemophilia A is characterized by a deficiency in ?

A

Factor VIII:C

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8
Q

What is the therapy done in Hemophilia A?

A

Commercial VIII concentrate
DDAVP

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9
Q

This will increase the factor VII inside the body and will allow the release of stored vWF?

A

Desmopressin

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10
Q

Desmopressin will increase the________ inside the body and will allow the release of stored ______?

A

Factor VII
vWF

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11
Q
A
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12
Q

This can exhibit mild hemophilia A

A

Normandy vWF

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13
Q

Hemophilia B is AKA?

A

Christmas disease
Royal disease

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14
Q

The hemophilia B is AKA royal disease because?

A

It is because of queen victoria

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15
Q

This is a sex linked disorder characterized by a deficiency of factor IX?

A

Hemophilia B

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16
Q

Hemophilia B is a sex linked disorder characterized by a deficiency of what factor?

17
Q

Who is the first person to have hemophilia B?

A

Stephen christmas

18
Q

This is the second most common hemophilia, exhibiting clinical signs and symptoms similar to A?

A

Hemophilia B

19
Q

What is the therapy done in Hemophilia B?

A

Factor IX concentrate

20
Q

What is the result of the coagulation test in hemophilia A and B?

A

Both PT and APTT are prolonged

21
Q

What is the confirmatory test used in hemophilia B?

A

Factor IX assay

22
Q

What is the other name of hemophilia C?

A

Rosenthal syndrome

23
Q

Hemophilia C is AKA?

A

Silent type of hemophilia

24
Q

This is an autosomal recessive disorder characterized by a deficiency in factor XI?

A

Hemophilia C

25
26
Hemophilia C is an autosomal recessive disorder characterized by a deficiency of what factor?
Factor XI
27
Hemophilia C is increased among?
Ashkenazi Jews
28
What type of hemophilia is this: Bleeding is mild unless stressed by trauma or injury?
Hemophilia C
29
What is the therapy done in hemophilia C?
Fresh frozen plasma or whole blood
30
These are autosomal recessive disorders with no associated bleeding tendencies?
Prekallikrein
31
What is the coagulation result of hemophilia C and Prekallikrein?
PT is prolonged
32
What is the rarest deficiency in Prekallikrein?
Factor I deficiency
33
What is the most profound deficiency in prekallikrein?
Factor VII deficiency
34
Factor VII is most profound because of what reasons?
Presence of proteinemia Increase level of immunoglobulins in the blood
35
What is the reason why there is an increase of immunoglobulins in blood?
Increase viscosity of the blood Lead to kidney damage