Consideration of Splenectomy in Unstable Hemoglobinopathy: A Case Report of Hb Hammersmith (HBB: c.128T>C)

A 5-year-old female has been diagnosed with Hb Hammersmith (HBB: c.128T>C) and has required three blood transfusions thus far, with hemoglobin (Hb) levels dropping as low as 5.4 g/dL. An elective splenectomy is now being considered in order to reduce hemolysis and the need for transfusions. Of 18 previously reported cases of Hb Hammersmith, eight patients have reportedly undergone splenectomy, with only four of those studies reporting clinical improvement. Therefore, the role of splenectomy in unstable hemoglobinopathies remains unclear, but seems to be a promising option in Hb Hammersmith.