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Severity of disease |
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Depending on factors level
< 1% Severe, spontaneous bleeding
1-5% Moderate bleeding with minimal trauma or surgery
6-30% Mild bleeding with major trauma or surgery
Majority of patients - < 5% |
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Diagnosis |
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Shortly after birth
Profuse bleeding at circumcision.
Young child with moderate disease may not bleed until they begin to crawl or walk.
Pt presents with pain followed by swelling in weight bearing joint such as knee, hip or ankle.
Hemarthrosis- inflammation of synovial membrane
Repetitive episode erodes articular cartilage and causes osteoarthritis, articular fibrosis, joint ankylosis, eventually muscle atrophy. |
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Laboratory Diagnosis |
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PTT – prolonged
PT
Platelet count – normal or increased
Bleeding time
Factor assay
VWF assess – to differentiate between vWD and Hemophilia, to identify carrier status |
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Complications |
|
| 1 |
Hepatitis |
| 2 |
Elevated hepatocellular enzymes |
| 3 |
Abnormality on liver biopsy |
| 4 |
Despite of frequent bleeding, iron deficiency anemia is uncommon. |
| 5 |
Following multiple transfusion, 10 to 20% pt with severe hemophilia develop inhibitors to FVIII |
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Treatment |
|
| 1 |
Indications |
| 2 |
Prophylaxis Vs On demand |
| 3 |
Joint/ Muscle bleed |
| 4 |
Surgery |
| 5 |
Plasma product rich in F VIII |
| 6 |
Cryoprecipitate |
| 7 |
Lyophilized F VIII concentrate |
| 8 |
Monoclonal Purified F VIII |
| 9 |
Recombinant F VIII |
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