Abstract
Hyperhemolysis syndrome by Bystander mechanism in siblings with sickle cell disease
Highlights
Sickle cell anemia (SCA) is a genetic disorder characterized by homozygous hemoglobin S (Hb S), chronic haemolytic anemia and painful episodes
We aim to highlight the importance of recognizing such episodes as they present as pain crisis in SCD patients following transfusion and preventing wrong management of crisis with red blood cell (RBC) transfusion which can increase hemolysis and may cause life-threatening anemia
We report cases of bystander hemolysis in siblingswith SCA, 1 to 2 weeks after blood transfusion
Summary
Sickle cell anemia (SCA) is a genetic disorder characterized by homozygous hemoglobin S (Hb S), chronic haemolytic anemia and painful episodes. A 13 years old girl known case of SCD was referred to our hospital with pain crisis from her native town, where she was treated with analgesics, intravenous fluids, and two units of ‘O’ Rh D positive whole blood transfusion Pretransfusion reports from her native town showed hemoglobin (Hb) to be 8.2 gm%, Hematocrit (HCT) as 25% and bilirubin to be 2.7 mg/dl (unconjugated -1.9 mg/dl). After admission to the Paediatrics Department of our hospital, her blood test reports showed haemoglobin (Hb) level of 6.2 gm%, Hematocrit (HCT) of 20%, LDH of 943 U/L and Bilirubin 6 mg/dl (unconjugated -5.5 mg/dl) She was transfused with three units of ‘O’ Rh D positive packed red blood cells (PRBC) and was referred to our department after 10 days for low hemoglobin despite blood transfusions. Antibody screening and identification of the sister using ortho surgiscreen and resolve panel A
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