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A Life-threatening Post-transfusion Reaction in an Individual With Sickle Cell Disease

Hematopoiesis Case StudiesA 57-year-old African American woman living with sickle cell disease (SCD; SS genotype) and baseline hemoglobin (Hgb) of 6.0 to 7.0 g/dL, presented to the emergency department (ED) with shortness of breath and pain consistent with a vaso-occlusive crisis. Her Hgb level was 6.3 g/dL; she was treated with supportive measures and received one unit of phenotypically matched packed red blood cell (PRBC) transfusion for symptomatic anemia. She showed improvements and was subsequently discharged.

She returned to the hospital eight days later with progressive fatigue, decreased oral intake, and dark urine. Initial evaluation revealed tachycardia (heart rate, 110/min), and physical examination was notable for scleral icterus. The rest of the physical exam was unremarkable.

Initial laboratory findings showed the following:

Test Result Range
Hgb 2.8 g/dL 12-15 g/dL
Hematocrit 8.3% 36-46%
RBC count 0.87 M/µL 4.15-5.55 M/µL
Mean corpuscular volume 95.5 fL 80-100 fL
White blood cell count  28.4 K/µL 3.8-10.6 K/µL
Platelet count 125 K/µL 150-450 K/µL
Haptoglobin < 30 mg/dL 30-200 mg/dL
Lactate dehydrogenase 3,420 IU/L < 250 IU/L
Ferritin 1,211 ng/mL 11-307 ng/mL
Total bilirubin  2.7 mg/dL < 1.2 mg/dL
Direct bilirubin 0.6 mg/dL 0-0.3 mg/dL
Reticulocyte count 3.5% 0.5-1.5%
Fibrinogen 263 mg/dL 200-450 mg/dL
D-dimer > 20 µg/mL < 0.50 µg/mL
Prothrombin time 19.8 seconds 11.5-14.5 seconds
Activated partial thromboplastin time 32 seconds 22-36 seconds
Antibody screen Negative  
Direct antiglobulin test Negative  
High sensitivity Troponin 650 ng/L < 19 ng/L

  1. Gardner K, Hoppe C, Mijovic A, et al. How we treat delayed haemolytic transfusion reactions in patients with sickle cell disease. Br J Haematol. 2015;170:745-756.
  2. Chadebech P, Habibi A, Nzouakou N, et al. Delayed hemolytic transfusion reaction in sickle cell disease patients: evidence of an emerging syndrome with suicidal red blood cell death. Transfusion. 2009;49:1785-1792.
  3. Coleman S, Westhoff CM, Friedman DF, et al. Alloimmunization in patients with sickle cell disease and underrecognition of accompanying delayed hemolytic transfusion reactions. Transfusion. 2019;59:2282-2291.

Drs. Sweidan, Vuyyala, Otrock, and Dabak indicated no relevant conflicts of interest.