I am caring for a 1 9/12 y old girl with sickle cell anemia, and now with splenic sequestration. Is chronic transfusion still the treatment of choice in this age group?
generally, yes. There does not appear to be a consensus of expert practice, but three general approaches. Some experts proceed to splenectomy at this age, others wait to allow a bit of additional maturation of the immune system before splenectomy, and others are trying to find ways to preserve some spleen immune function by performing partial splenectomy. At our center we would put such a patient on monthly transfusion if there has been one life-threatening splenic sequestration or two episodes of splenic sequestration symptomatic enough to require RBC transfusion. Monthly transfusions would not have a specific hemoglobin goal, nor a target percentage of HbA. Instead, the therapeutic goal is to provide some amount of normal RBC so that acute splenic sequestration will not have a life-threatening recurrence. Splenomegaly usually persists on this transfusion program, and the spleen can fluctuate in size with some dips in the Hb and platelet count, but without symptoms. Our timing approac
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