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Catastrophic Delayed Hemolytic Transfusion Reaction in a Patient With Sickle Cell Disease Without Alloantibodies

Case Report and Review of Literature

Jasinski, Sylwia MD; Glasser, Chana L. MD

Journal of Pediatric Hematology/Oncology: August 31, 2018 - Volume Publish Ahead of Print - Issue - p
doi: 10.1097/MPH.0000000000001307
Clinical and Laboratory Observations: PDF Only

While packed red blood cell (PRBC) transfusion therapy is a mainstay in the treatment of certain patients with sickle cell disease (SCD) and the standard of care for preoperative management, there are associated risks. Delayed hemolytic transfusion reaction (DHTR) is a risk of PRBC transfusion occurring 2 to 20 days from transfusion and typically presents with severe pain characteristic of vaso-occlusive crisis, fever, and hemolytic anemia. DHTRs are uncommon, occurring in only 4% to 11% of transfused patients with SCD, but may be catastrophic in nature with progression to multiorgan failure within hours. Here, we describe a case of a 20-year-old female with sickle cell SS disease who developed a severe DHTR 5 days following an elective preoperative PRBC transfusion, and rapidly progressed to multiorgan failure and death. This is the first reported case of a catastrophic DHTR in a patient with SCD without any detectable known or new alloantibodies.

Department of Pediatric Hematology/Oncology, NYU Winthrop Hospital, Mineola, NY

The authors declare no conflict of interest.

Reprints: Sylwia Jasinski, MD, Department of Pediatric Hematology/Oncology, NYU Winthrop Hospital, 120 Mineola Boulevard, Suite 460, Mineola, NY 11501 (e-mail:

Received February 28, 2018

Accepted August 12, 2018

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