A 10 year-old male admitted with symmetric swelling of upper thighs, with tenderness. The swelling had appeared spontaneously.
The
patient has a history of hemophilia A.
He had a history of numerous episodes of bleeding into the knees, ankles
and elbows. He had been hospitalized on
several occasions.
A
number of male relatives on the maternal side of the family had been diagnosed
with hemophilia A.
In addition to the swelling in the upper thighs, both knees were also noted to be swollen. The patient was pale and in distress.
PT=
10 sec (Normal 8-11.5)
aPTT > 100 sec (Normal 24-37.5)
Plt=
490,000 /μL (Normal 150,000-350,000)
OTHER PERTINENT
LABORATORY RESULTS
Hgb=6.0 g/dL (Normal 13-18)
HOSPITAL COURSE
Upon
admission, the patient received packed red cells, which increased the
hemoglobin level to 10 g/dL. In
addition, he received 2,000 units of Factor VIII concentrates. The aPTT measured 2 hours after infusion was
greater than 100 sec. Factor VIII activity was less than 1%.