The patient was first seen in our emergency department at
the age of 7 years with petechiae, ecchymoses, and intermittent,
atraumatic high-volume epistaxis from both nares that is worsened with
nasal manipulation, His initial platelet count was
26,000/mm3. He had microcytic hypochromic anemia,
presumably due to iron deficiency caused by recurrent epistaxis. The
bleeding was controlled in the ED with local measures and aminocaproic
acid.
During outpatient visit, the family was counseled and 25
mg of eltrombopag daily was started. Two weeks later the dose was
increased to 50 mg daily due to persistent symptoms following which the
family reported significant symptomatic improvement. In light of lack of
platelet count response, we stopped eltrombopag and asked family to keep
a bleeding journal. Within 4 days of stopping eltrombopag, the patient
experienced a large nosebleed which was managed with local measures and
aminocaproic acid. The patient experienced recurrent epistaxis in the
ensuing weeks. Family insisted on restarting eltrombopag based on their
observation of clinical response and eltrombopag was resumed at 50 mg
daily. Within 2 weeks, family again reported reduced epistaxis and
resolving bruises. The patient is currently stable on 50 mg Eltrombopag
daily remaining asymptomatic/minimally symptomatic. (See Figure 1,
epistaxis scoring was calculated using ISTH/SCC bleeding assessment
tool. Epistaxis scoring was calculated using ISTH/SCC bleeding
assessment tool 15). Mean platelet
volume (MPV) has been inconsistent ranging between 6.3 fL and 16 fL
without correlation with symptoms or treatment.