Hemophelia Flash Cards

Front 1

Definition of Hemophelia

Back 1

a blood coagulation disorder caused by the deficiency of absence in one of the plasma clotting factors

Front 2

Hemophilia A is a deficiency in which clotting factor?

Back 2

Clotting factor VIII

Front 3

Hemophilia A is a deficiency in which clotting factor?

Back 3

VIII

Front 4

Hemophilia B is a deficiency in which clotting factor?

Back 4

IX

Front 5

What are the 3 protein deficiencies that can cause Hemophilia?

Back 5

VIII, IX, vWF

Front 6

What is the most common inherited bleeding disorder?

Back 6

von Willebrand disease

Front 7

Most of Hemophiliacs have which type of Hemophilia?

Back 7

(85%) Hemophilia A, deficiency in Factor VIII

Front 8

What are the 3 classifications of Hemophilia?

Back 8

Severe, moderate, mild

Front 9

Describe severe hemophilia...

Back 9

-inherent activity of clotting factors is <1% of normal
-spontaneous bleeds occur every 1-6 months
-now diagnosed as infant

Front 10

Describe moderate hemophilia...

Back 10

-inherent activity of clotting factors is 1-5% of normal
-bleeding follows min to mod trauma
-diagnosed at 1-2yo
-more frequent bleeds occur 3-4yo

Front 11

Describe mild hemophilia...

Back 11

-inherent activity of clotting factors is 5-30% of normal
-bleeding following major surgery/trauma
-may go undiagnosed 2-adulthood

Front 12

How many factors are involved in the clotting cascade?

Back 12

20

Front 13

Explain important components of the clotting cascade

Back 13

1. damage to the endothelium causes release of vWF, collagen, and TF.
2. vWF calls in Factor VIII and platelets
3. Collagen and vWF change platelet structure and cause release of granule contents--> alpha, dense granules
4. Factors follow cascade ending by converting prothrombin-->thrombin which converts fibrinogen to fibrin.
5. fibrin catches lipids and platelet debris and forms clot
FIBRINOGEN= key

Front 14

When collagen and vWF bind to platelets and cause release of contents from granules, what is released by the alpha granule?

Back 14

factor 4,5,8
growth factor
fibrinogen
vWF

Front 15

When collagen and vWF bind to platels and cause them to release the contents of their granules, what is released from the dense granule

Back 15

ADP
ATP
Serotonin
Ca++

Front 16

Who is the "general" who calls in the components of the clotting cascade? And where is it made?

Back 16

vWF
endothelium of vasculature

Front 17

Where are clotting factors made?

Back 17

in the liver except for
factor 8 which are made in endothelium
vonWillebrand's factor which is made in the endothelium, α-granules of platelets, and subendothelial connective tissue.

Front 18

According to the CDC what is the definition of a target joint?

Back 18

A joint in which 4+ bleeds have occurred in the last 6mo OR in which 20 bleeds have occurred in a lifetime.

Front 19

PTT measures which clotting pathway?

Back 19

Extrinsic
TF--> Thrombin
approx 12-15 sec, varies by location
HEPRIN

Front 20

aPTT measures which clotting pathway?

Back 20

Intrinsic (longer)

Front 21

Key in initiating the intrinsic pathway?

Back 21

Fibrinogen

Front 22

Where are platelets synthesized?

Back 22

Bone marrow.

Front 23

Mechanism of action of Anabolic Steroids?

Back 23

release contents of granules
Alpha granules-GF=bulk up
dense granules- ATP ENERGY

Front 24

Most common "target joint"?

Back 24

44%knee
ankle
elbow
(large synovial, more at risk if WB joint)

Front 25

Cause of synovitis?

Back 25

Hemearthrosis causes irritation of the synovium secondary to iron deposits in synovial membrane. Synovitis causes neovascularization increasing risk of future bleeds

Front 26

Common Consequences of bleeding disorders:

Back 26

Joint scarring
Vision loss (severe case)
Chronic Anemia
Neurological/ psychiatric problems

Front 27

Signs/ symptoms of iliopsoas hemorrhage?

Back 27

can't see it
-pain in hip and abdomen area
-overactive bladder (bladder issues)

Could turn into life threatening retro-peritoneal bleed.

Front 28

Effects of chronic Intra-muscular bleeds?

Back 28

atrophy, fibrosis, contracture, pseudotumor formation.

Front 29

What kind of genetic disorder is hemophilia?

Back 29

x-linked autosomal recessive

Front 30

Why would liver disease cause a clotting disorder?

Back 30

it is where clotting factors are synthesized

Front 31

Why would bone marrow disorders cause bleeding disorders

Back 31

platelets are formed in the bone marrow

Front 32

When pts have inhibitors that attack factors in the body, what are some options available for treatment?

Back 32

Immune Tolerance Therapy
Porcine Factor (from pig)

Front 33

When using whole blood in the 60's and 70's to replace factor deficits-- what were hemophiliacs exposed to?

Back 33

Hep A
Hep B
Hep C
HIV

Front 34

When was the worst AIDs outbreak in the hemophiliac population?

Back 34

The 80's

Front 35

When did research in gene therapy and recombinant factor developed?

Back 35

90's

Front 36

What are some other clotting disorders?

Back 36

Factor V Leiden (hypercoag)
Protein C deficiency
Protein S deficiency
Anti-thrombin deficiency
Pro-Thrombin Mutations
DVT

Front 37

Hemophilia Treatment Centers are required to have which medical professionals on staff?

Back 37

Orthopedist
Hematologist
PT
Nurse
Social Worker

Front 38

Medical Treatment options for Hemophiliacs include:

Back 38

Factor VIII and IX concentrations
Recombinant factor of VIII and IX
Frozen Plasma
DDAVP- Desmopressin Assetate: (mimics vasopressin, decrease kidney excretion and increase factor release into blood stream)
Animal Factor
Prothrombin Complex Concentrates
Prophylaxsis

Front 39

Indications for a joint replacement in a hemophiliac?

Back 39

Pain, >20 degrees loss ROM
Wait if possible since prosthesis only last 10 years (haven't improved since the 60's.

Front 40

When do you want to exercise post factor therapy?

Back 40

Early, low impact--> Aquatics