Follow-up
At six-month, one-year, and two-year follow-up, the patient continued to
do well with no new bleeding events. FVIII inhibitor remained
undetectable and FVIII activity was persistently normal. He was
discharged from hematology after two years of follow-up with apparent
resolution of AHA.
Case 2
The second patient was a 11 year-old previously healthy female who
presented to the hematology clinic for joint pain and increased
bruising. Symptoms began shortly following routine influenza
vaccination, when she developed right calf swelling and pain associated
with decreased range of motion of her right knee. There was no known
trauma to the area, and initial workup was reportedly unremarkable. Over
the next two weeks, she experienced swelling and bruising of both
forearms unrelated to any trauma. She did not have any personal or
family history of significant bleeding disorders.
The patient first presented to hematology clinic one month after symptom
onset, and physical examination revealed bilateral forearm ecchymoses,
swollen and painful wrists and elbows bilaterally, swelling and
ecchymosis of the right calf, and a large effusion of the right knee.
Investigations revealed a prolonged PTT (66.5 sec), FVIII activity 7.2%
and a FVIII inhibitor (3.8 B.U.; Fig. 2). She was hospitalized and
treated with rFVIII 50 U/kg twice daily and prednisone 1mg/kg twice
daily. She received three doses of recombinant FVIII with minimal
improvement in her symptoms so was switched to rFVIIa. Her soft tissue
bleeds markedly improved with rFVIIa and she was discharged home.
The patient received prednisone 1 mg/kg/dose twice daily as an
outpatient for ten days and experienced continued symptom improvement,
normalization in FVIII activity (104%) and declining inhibitor levels
(0.4 B.U.). Steroids were gradually tapered over the course of six
weeks, during which time there were no new bleeding events, FVIII
activity remained normal, and FVIII inhibitor levels remained
undetectable.