Female adolescent with recurrent anemia and thrombocytopenia: Questions

Excerpt

A 14-year-old Caucasian female with a strong family history of autoimmune diseases presented with dyspnea and a new-onset petechial rash to the emergency department (ED). One month prior to presentation, she had an episode of bloody diarrhea that self-resolved. Physical exam, including vital signs, was unremarkable except for diffuse pallor and petechiae without lymphadenopathy or organomegaly. Weight on admission was 102.3 kg. Her initial labs were remarkable for anemia (Hgb 7.9 g/dL), thrombocytopenia (23 cells/ × 10⁹L), increased LDH (454 U/L), hyperbilirubinemia (total bilirubin 2.2 mg/dL), and undetectable haptoglobin. Direct antibody test (DAT) was positive for warm antibodies against immunoglobulin (Ig)G and C3. Peripheral blood smear demonstrated rare spherocytes, but no schistocytes. Additional labs evidenced normal kidney function, negative enzyme immunoassay (EIA) for Shiga-toxin in stool, ADAMTS13 activity of 100%, and negative antinuclear and antiphospholipid antibodies. The patient was diagnosed with Evans syndrome (ES) and admitted to the hematology floor for treatment. She was treated with prednisone (1 mg/kg/day) and remained hospitalized for 3 days. After 1 week of treatment, her hemoglobin improved (10.8 g/dL) and her platelet count normalized (213 cells/ × 10⁹L). After 2 weeks, a slow prednisone taper was initiated and a more rapid taper ensued after 1 month due to the onset of significant side effects such as steroid-induced diabetes requiring insulin, and weight gain. …