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40 Cards in this Set

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Synonyms for Hemophilia A
and Percentage
Classical Hemophilia
Factor VIII Deficiency
(85%)
Synonyms for Hemophilia B
and Percentage
Factor IX Deficiency
Christmas Disease
(10%)
Why do you have bleeding with hemophilia?
Because you do not get amplification of the coagulation cascade in the intrinsic pathway
Which lab value is prolonged in hemophilia?
PTT b/c instrinsic pathway
What intron inversion is found in a large population of patients with Hemophilia A?
Inversion 22 (genetically test for this first
What would you genetically term the daughter of a male hemophiliac?
an obligate carrier
Is it possible for a female to have hemophilia?
- lionization
- parents are male with disease and carrier female
How much hemophilia is due to spontaneous mutations?
30%
What are the clinical manifestations of Hemophilia?
- Joint bleeds --> Target joints (MOST COMMON is large joints)
- Deep muscle bleeds
- Hematomas
- Post-surgery/Post-trauma
- Dental extraction
- Intracranial hemorrhage
Can female hemophilia carriers have symptoms?
Yes. Because they have decreased Factor VIII.
menorrhagia
postoperative bleeding
postpartum bleeding
epistaxis
oral, dental bleeding
target joint
repeated bleeding into the same joint
Can hemophilia skip generations?
Yes. If 2nd generation is a carrier
What drugs should hemophiliacs avoid?
Aspirin
NSAIDs
Severe Clinical Manifestations
Hemophilia
<1% Clotting Activity
Early childhood bleeding
Spontaneous bleeds
Moderate Clinical Manifestations
Hemophilia
1-5% Clotting Activity
After minor trauma or surgery, pt bleeds
May be spontaneous bleeding
Mild Clinical Manifestations
Hemophilia
5-20% Clotting Activity
Bleeding after trauma only
HEMOPHILIC ARTHROPATHY
- Recurrent bleeds leading to persistent inflammation, soft tissue, bone destruction
- Recurrent pain, chronic arthritis
- Decreased range of motion and mobility
- Eventual physical impairment, long-term disability, psychosocial consequences
Synovitis
- blood damages the synovium
- if synovitis persists then you get muscle wasting, morning stiffness, chronic pain, limited movement
MRI in Hemophilic Arthropathy
- Knee effusion
- Synovial hyperplasia
- Ankle effusion
INTRAMUSCULAR/SOFT TISSUE BLEEDING
in Hemophilia
Less common than hemarthroses, but may have more severe consequences. Areas of greatest concern include:
* Palm of hand, wrist
* Calf
* Thich or Groin
- Face, neck, mouth, eye
- Hip of shoulder
- Laceration requiring sutures
- Hematuria, GI Tract Bleeding
* Potential Circumferential Compression
Intracranial hemorrhage
in Hemophilia
- MOST FEARED COMPLICATION
PFA100
Platelet Function Analysis 100
- take cold, anticoagulated blood
-pull it through a membrane by capillary action and see how long it takes for a clot to form
- THINK OF IT AS AN EX-VIVO BLEEDING TIME
What will prolong your PT and not PTT?
Factor VII deficiency
Treatment of Hemophilia
- Hemophilia Treatment Centers
- Plasma derived or Recombinant factors to replace the missing factor
- Clotting factor concentrates are given to prevent bleeding and to limit existing hemorrhage
- Synovectomies, joint replacements/fusions
- PREVENTION, PREVENTION, PREVENTION
How do you prevent bleeding in Hemophilia?
Administer Recombinant Factors 2-3 times/week
How do you obtain plasma-derived concentrates (that will be used for Hemophilia Treatment)?
-large starting pool of carefully screened donor plasma
-Affinity chromatography using monoclonal antibodies
-Viral inactivation procedures (pasteurization, solvent-detergent treatment, ultrafiltration) are effective against HIV and hepatitis viruses.
How do you obtain Recombinant human factor VIII and IX (that will be used for Hemophilia Treatment)?
- purified from the cell culture of transfected mammalian cell lines
- require no further viral attenuation
Complications of Hemophilia Treatment
- Development of inhibitors (alloantibodies) to Factor VIII and less commonly, Factor IX (about 10-20% develop this)
- Infectious
- Hep B (70-90% prior to vaccine)
- Hep C (>90% in pts treated prior to 1985)
- HIV (By 1984, >90% of severe Hemophilia A)
- CMV, Parvovirus B19
At what age to begin hemophilia prophylaxis?
1-2 years old begin with IV administration
Acquired Hemophilia
- development of neutralizing and clearing anti-factor VIII (FVIII) antibodies in individuals without a preexistent congenital FVIII deficiency.
- uncommon - incidence of 0.2 to one cases per million population per year
- Older adults
- Postpartum
- 50% with underlying autoimmune DO (lupus, rheumatoid arthritis), malignancy
- 50% idiopathic
What is the most common inherited bleeding disease?
Von Willebrand's Disease
Von Willebran's Disease
- Heterogeneous group of inherited or acquired bleeding disorders
- Bleeding due to reduced level or abnormal function of VWF
- Most types have autosomal dominant inheritance
What is the life of Factor VIII if not bound to vWF?
8 hours
Clinical Bleeding Symptoms Associated with Type 1 Von Willebrand Disease
- Gingival Bleeding
- Hemorrhage after dental extraction
- Epistaxis
- Post-operative bleeding
- GI bleeding
- Joint bleeding (will look like platelets and hemophilia bleeding)
- Menorrhagia
- Hematuria
- Post-partum Bleeding
Do pts with Von Willebrand's Disease have problems with primary or secondary bleeding?
BOTH
What are the laboratory findings in von willedbrans disease?
PTT - prolonged or normal
Factor VIII - may be moderately reduced
VWF - low
DDAVP
(1-deamino-8-D-arginine vasopressin = synthetic analog of ADH L-vasopressin)

Releases stored Factor VIII and VWF from endothelial cells

- this causes a quick response, but cannot be used repeatedly b/c you do not have more stores
Humate P
- Intermediate purity factor VIII concentrate which contains both VWF and factor VIII

FOR PTS WITH SEVERE VWD
Treatment of VWD
- DDAVP
or
- Humate P
AMICAR
- helps stabilize the fibrin clot