Chapter 16 Hemophilia

Question 1

How can hemophilia present in a five-year-old boy?

Answer 1

ProlongEd oozing of blood days after tooth extraction

Question 2

What are the differential diagnosis for hemophilia symptoms?

Answer 2

Primary hemostasis (vasculitis, thrombocytopenia, and qualitative platelet disorders) and secondary hemostasis (clotting factor deficiencies)

Question 3

What are common symptoms in secondary hemostasis?

Answer 3

Delayed bleeding, ecchymoses, Hematoma in joints and muscles

Question 4

What are the diagnostic labs for diathesis?

Answer 4

CBC PTT PR BMP bleeding time, Factor VIII level

Question 5

What inherited causes result in prolonged PTT with normal PT? Acquired?

Answer 5

Deficiency in factor 8, 9, 11, von Willebrand factor disease; Antiphospholipid syndrome and Heparin use

Question 6

We should be done if patient has isolated prolonged PTT?

Answer 6

Obtain the levels of intrinsic clotting factors (Factor eight, nine, 11)

Question 7

How should this patient be treated?

Answer 7

Administer purified monoclonal recombinant factor eight (hemophilia a) or factor nine (hemophilia B).
Desmopressin and anti-fibrinolytic agents if necessary

Question 8

What is the appropriate counseling for hemophilia a?

Answer 8

Patient, family, genetic counseling and no contact sports and no aspirin use

Question 9

We should be monitored for these patients?

Answer 9

PTT and or factor eight plasma

Question 10

When should therapy be initiated for hemophilia a patients?

Answer 10

Right after obtaining Factor VIII levels

Question 11

When should the patient be transferred to the ward?

Answer 11

When the PTT results come back

Question 12

When should we consult genetics along with other counseling?

Answer 12

After you revaluate the patient every 1 to 2 hours and PTT along with factor eight is within normal range