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.