An 18-year-old man with known hemophilia A is examined in the emergency departme

Question

An 18-year-old man with known hemophilia A is examined in the emergency department for knee pain and swelling. He is given a dose of factor VIII concentrate appropriate for his plasma volume and target factor VIII level.

Factor VIII activity before treatment: 1%

Factor VIII activity after treatment: 2%

1. Is this an acceptable increase in factor activity after treatment?

2. What is the most likely cause of unexpectedly low factor VIII activity after such therapy?

3. What additional tests should be performed?

The results of the proposed additional studies were as follows:

aPTT: 96 sec

Mix of patient plasma and normal plasma (1:1):

Immediate: 39 sec

After 2-hour incubation (37° C): 94 sec

Bethesda titer: 4 units

4. Based on all the data provided, what condition is most likely?

5. What is the treatment for patients with this condition?

Explanation / Answer

Ans 1..No the.increase is not.appropriate

For hemostasis to occur minimmm factor 8 activity should be 25.percent

Ans 2..There may be the following reason of abnormal low level favtorf8 activity

DiC...Disseminated intravascular coagulatiom

Von villibrand disease

The patient may be on some.blood thinner like asprin

But even after the therapy low.level indicates presence of Inhibitors.

Ans.3...AAdditionnal test that should be performed are

aPTT

Prothrombin tim3

Platelet count

Bleeding time

Clotting time

Bethesda Assay

Ans 4....The most likely condition is DIC.the factor must have exhausted due to which there is continuous bleeding and factor 8 level are also not increasing due to DIC

Related Questions

Navigate

• Browse (All)
• Browse A
• Subjects

Integrity-first tutoring: explanations and feedback only — we do not complete graded work. Learn more.