Red cell exchange for acute splenic sequestration crisis in an adult with hemoglobin sc disease
Omosule A, Otrock ZK. Red cell exchange for acute splenic sequestration crisis in an adult with hemoglobin sc disease. Journal of Clinical Apheresis 2018; 33(2):196-197.
Journal of Clinical Apheresis
Purpose Acute splenic sequestration crisis (ASSC) is a well-known life-threatening complication of sickle cell disease in children. It is characterized by progressive splenic enlargement, severe anemia requiring multiple transfusions, and ultimately circulatory compromise. Modalities of management include red blood cell (RBC) transfusion, pain control, and splenectomy. ASSC has rarely been reported in adults, thus making its management and outcome in adults not clearly defined. The purpose of this report is to describe the favorable outcome of ASSC in an adult female with hemoglobin (Hb) SC disease managed with RBC exchange, thus avoiding a scheduled splenectomy. Methods Case report and literature review Results A 39-year-old African American lady with history of Hb SC disease, diagnosed at age of 10, presented to the surgical outpatient clinic with 1-month history of progressive abdominal swelling, abdominal pain and easy satiety. She was evaluated by her hematologist and was found to have markedly enlarged spleen on imaging measuring 20cm in its longest dimension raising the suspicion for ASSC. As a result, she has been given multiple blood transfusions over the past month with transient mild improvement of symptoms. Her past medical history was significant for a similar episode of abdominal pain at the age of 16, which responded to blood transfusion and pain management. Splenectomy was recommended in light of persistence of patient's symptoms despite management with transfusions and pain medications. In preparation for splenectomy, the transfusion medicine service was consulted to perform one session of RBC exchange few days before surgery. Patient's laboratory values on admission were Hb 8.7 g/dL, hematocrit 25.9%, WBC 3.8 × 109/L, MCV 81.2 fL, and platelets 105 × 109/L. Hb electrophoresis analysis revealed a combined Hgb S and C of 91%. RBC exchange was performed using the COBE Spectra apheresis system (COBE Spectra, Terumo BCT, Inc., Lakewood, CO) processing 2 blood volumes and replacing with fresh (<10 days old), leukoreduced, sicklecell negative, C, E and K antigen-negative packed RBCs. We aimed for end hematocrit of 30% and end Hb S1C of 20%. We thought that the patient might respond to RBC exchange, so we recommended holding on splenectomy to evaluate the patient for response to RBC exchange. The patient tolerated RBC exchange very well; however her post procedure period was complicated by hypotensive episodes which were responsive to adequate hydration. Repeat evaluation after exchange revealed Hb 9.1g/dl and Hb electrophoresis showed Hb S1C of 29.8%. The patient started to feel better after RBC exchange and she could eat without discomfort. Her spleen was barely palpable below the left costal margin, and there was a marked reduction in splenic size on repeat imaging to 17cm four days after exchange. At 2-month follow up, the patient was free of symptoms and she did not require further blood transfusions. She was being considered for long term hydroxyurea 1/2 RBC exchange as outpatient. Conclusion Red cell exchange is used to replace sickle cells with normal RBCs. There are rare case reports on the role of exchange transfusion in adults with Hb SC disease. RBC exchange should be considered in adults with Hb SC disease with ASSC; a procedure that could improve patient outcome and avert complications from splenectomy especially in young patients.