Question 6: Were any red blood cell (RBC) transfusions administered?

Red blood cell (RBC) transfusions are often given as supportive care to lower hemoglobin S for recipients diagnosed with Sickle Cell Disease (SCD). If the recipient received any RBC transfusions between diagnosis and the start of the preparative regimen / infusion, select Yes. If the recipient did not receive any RBC transfusions or no information is available to determine if the recipient received RBC transfusions, report No and go to question 17.

Question 7: Number of RBC transfusion events within the last 12 months

Transfusions may be referred to as “simple” or “exchange” transfusions. A simple transfusion refers to a direct infusion of a blood product. An exchange transfusion refers to the slow removal and replacement of the recipient’s blood with that of a healthy donor’s blood. A transfusion event consists of one or more RBC unit(s) given in a day.

Indicate the total number of RBC transfusion events the recipient received within 12 months prior to the start of the preparative regimen / infusion to prevent sickle-related vaso-occlusive events.

Example: The progress notes state a recipient was transfused with one RBC unit for each month, for six months. The number of transfusions increased, and the recipient receives two RBC units on the same day, each month, for the following six months prior to the start of the preparative regimen. The total number of RBC transfusion events within the last 12 months would be reported as “12.”

Question 8: Date of last transfusion administered

Report the date of the most recent RBC transfusion administered prior to the start of the preparative regimen / infusion.
If the last administration date is partially known (i.e., the recipient received their last RBC transfusion in mid-July 2019), use the process described for reporting partial or unknown dates in General Instructions, Guidelines for Completing Forms.

Question 9: Was a transfusion administered to PREVENT sickle cell related events? (includes simple or exchange transfusions)

Transfusions administered routinely to offset the effects of sickle cell-related events are often called “chronic transfusions” within progress notes. In the context of reporting, “chronic transfusions” are defined as eight or more transfusion events per year, for at least a year, or a lifetime cumulation of ≥ 20 transfusion events between diagnosis and referral for transplant.

If any of the red blood cell transfusions were administered to prevent sickle cell related events, report Yes.

Report No in the following scenarios:

  • If the recipient did not receive transfusions to prevent sickle cell related events.
  • There is no information available to determine if the recipient received transfusions to prevent sickle cell-related events.
  • If the recipient is receiving transfusion therapy for reasons other than to prevent sickle cell-related events.

Questions 10 – 11: Date transfusion started

Indicate if the date transfusion(s) administered to prevent sickle cell related events began is Known or Unknown. If Known, enter the date when the first transfusion began in question 11. If transfusions were given over multiple days, report the first date of administration where the intent of transfusion was to offset or prevent the effects of sickle cell-related events. The reported start date should align with the recipient’s sickle cell-related event for which the transfusion was administered and may not be the same transfusion event reported in question 8. If the reported transfusion start date is an estimated date, also select Date estimated.

If the therapy start date is not known, report Unknown and go to question 12.

If the start date is partially known (i.e., the recipient started treatment in mid-July 2019), use the process described for reporting partial or unknown dates in General Instructions, Guidelines for Completing Forms.

Questions 12 – 13: Date therapy stopped

Indicate if the transfusion therapy stop date is Known or Unknown. If the therapy stop date is Known, enter the date when the transfusion(s) to prevent sickle cell related events stopped in question 13. If the reported transfusion start date is an estimated date, also select Date estimated.

Report Unknown if the stop date is not known and go to question 14.

If the recipient is still receiving transfusions to prevent sickle cell related events at the start of the preparative regimen / infusion, report Not applicable and go to question 14.

If the stop date is partially known (i.e., the recipient stopped treatment in mid-September 2019), use the process described for reporting partial or unknown dates in General Instructions, Guidelines for Completing Forms.

Question 14: Are the RBC units used for transfusion of an extended phenotype match (D, C, c, E, e, K)? (includes partial extended phenotype matches)

Extended phenotype testing may be performed on RBC units prior to transfusion to ensure donor and recipient matches are confirmed beyond the standard ABO compatibility matching to decrease the risk of alloimmunization. This information is typically found within the blood bank section of the medical record.

Report Yes if the RBC unit(s) used for transfusion are of an extended phenotype match (particularly D, C, c, E, e, or K). If the RBC unit(s) used for transfusion were not matched for extended phenotype D, C, c, E, e, or K or it is unknown if matched, report No.

Questions 15 – 16: Are RBC alloantibodies present?

The presence of RBC alloantibodies may cause serologic incompatibility and make the selection of RBC units for future transfusions difficult. RBC alloantibodies are typically present once alloimmunization has occurred.

If RBC alloantibodies are present prior to the start of the preparative regimen / infusion, report Yes and specify the number of alloantibodies identified in question 16. If testing for RBC alloantibodies were performed multiple times prior to the start of the preparative regimen / infusion, report the most recent assessment.

If RBC alloantibodies were not present prior to the start of the preparative regimen / infusion, report No and go to question 17.

Report Unknown if testing was not performed or it is not known if alloantibodies were present and go to question 17.

Questions 17 – 18: Does the recipient have donor-specific antibodies present to the donor chosen for transplant? (Mean fluorescence intensity (MFI) > 1000 for HLA-A, HLA-B, and DRB1; and MFI > 2000 for HLA-C, DQB1, and DPB1 or positive virtual cross match)

Mean fluorescence intensity (MFI) is often used to determine the mean intensity and level of antibody expression within a sample. Donor-specific antibodies may be quantified through fluorescing techniques such as flow cytometry. This information may be found on an HLA report or within the blood bank section of the medical record.

Report Yes if the MFI > 1000 for HLA-A, HLA-B, and DRB1 or MFI > 2000 for HLA-C, DQB1, and DPB1 at any time prior to the start of the preparative regimen / infusion. If Yes, indicate if measures were taken to lower the MFI for the presence of donor antibodies prior to the start of the preparative regimen / infusion.

If testing was performed multiple times prior the start of the preparative regimen / infusion, report the most recent assessment.

Report No if the MFI ≤ 1000 for HLA-A, HLA-B, and DRB1 or MFI ≤ 2000 for HLA-C, DQB1, and DPB1 at any time prior to the start of the preparative regimen / infusion.

If testing was not performed to determine presence or absence of donor-specific antibodies, indicate Not tested. The Unknown option should be used sparingly and only when no information is available to determine if donor-specific antibody testing was conducted at any time between diagnosis and the start of the preparative regimen / infusion.

Last modified: Nov 11, 2020

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