All are true about hemophilia, except
|A||Increased Bleeding time (BT)|
|B||Decreased factor VIII|
|C||Decreased factor IX|
|D||Increased partial thromboplastin time (PTT)|
Increased Bleeding time (BT)
Please note that in hemophilia A factor VIII is reduced and hemophilia B factor IX is reduced. !!!
Reduction of factor VIII results in haemophilia A.
Factor VIII is synthesized by the liver primarily and also by spleen, kidney and placenta. It is bound to the von Willebrand factor (vWF)
Haemophilia is a X – linked disorder.
1. The normal factor VIII level is 50 – 100% (0.5 – 1.5 U/mL) and is usually measured by a clotting assay.
2. If factor VIII level is < 2%, patient presents with recurrent, spontaneous hemarthrosis which later leads to osteoarthritis, muscle hematomas involving calf and psoas muscles (it may lead to compression of femoral nerve and paraesthaesia in thigh and weakness of the quadriceps and contraction and shortening of the Achilles tendon).
3. If factor VIII level is 2 – 10%, mild trauma or surgery may cause haematomas.
4. If the factor VIII level is 10 –50%, major injury and surgery may cause excessive bleeding.
2. Muscle atrophy (due to haematomas)
3. Mononeuropathy (compression by haematomas)
4. Risk of hepatitis (B, C, D) and HIV through blood and blood product administration.
5. Pseudotumors syndrome
6. Pseudo phlebitis
7. Compartmental syndrome