Sickle Cell Disease: Transfusion Support
Patient Guideline Summary
Publication Date: January 27, 2020
This patient summary means to discuss key recommendations from the American Society of Hematology (ASH) for transfusion support for sickle cell disease.
- Note: sickle cell disease is an inherited genetic defect that causes red blood cells to deform under certain conditions. Those deformed cells plug small blood vessels, causing pain and tissue damage.
- Transfusions of blood or blood products can cause a potentially serious immune reaction. Symptoms include fever, chills, nausea, vomiting, diarrhea, itching, pain, feeling dread, and fainting blood in the urine.
- Transfusion reactions can ordinarily be prevented by blood type matching between the donor and the recipient, but multiple transfusions increase the risk to the recipient of blood-borne infection and iron overload and the difficulty of finding a fully matched donor.
- Standard donor matching is limited to ABO/RhD typing, but minor antigens – Rh (C, E or C/c, E/e) and K – can also cause mismatch reactions.
- We will use the abbreviation SCD throughout this summary to refer to sickle cell disease and ASH for the American Society of Hematology.
- This patient summary focuses on the management of transfusions in patients with sickle cell disease.
- The ASH recommends an extended red cell antigen profile by genotype (blood matching genes) or serology to include Rh (C, E or C/c, E/e) and K antigens as well as ABO/RhD for all SCD patients at the earliest opportunity, optimally before the first transfusion.
- Immunosuppressive therapy (intravenous immunoglobulin [IVIg], steroids, and/or rituximab) should be considered for all SCD patients at increased risk for a transfusion reaction.
- There are several available transfusion options depending upon individual patient circumstances such as pregnancy or a history of acute chest syndrome (ACS), which causes sudden chest pain, cough, fever, low oxygen levels, and lung damage:
- Simple transfusion
- Automated red cell exchange (RCE)
- Manual RCE
- Red cell exchange with isovolemic (no change in the patient’s blood volume) hemodilution (IHD-RCE)
- Regular or as-needed transfusions
- Transfusions before surgery
- The ASH guideline panel suggests iron overload screening by blood ferritin levels and magnetic resonance imaging (MRI) for liver iron content every 1–2 years in all patients with SCD receiving chronic transfusion therapy.
- ACS: Acute Chest Syndrome
- ASH: American Society Of Hematology
- DHTR: Delayed Hemolytic Transfusion Reaction
- IHD-RCE: Isovolemic Hemodilution
- MRI: Magnetic Resonance Imaging
- RCE: Red Cell Exchange
- SCD: Sickle Cell Disease
Chou ST, Alsawas M, Fasano RM, Field JJ, Hendrickson JE, Howard J, Kameka M, Kwiatkowski JL, Pirenne F, Shi PA, Stowell SR, Thein SL, Westhoff CM, Wong TE, Akl EA. American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support. Blood Adv. 2020 Jan 28;4(2):327-355. doi: 10.1182/bloodadvances.2019001143. PMID: 31985807; PMCID: PMC6988392.
The information in this patient summary should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.