Effects of Transfusion of Older Red Blood Cells on Patients With Hemoglobinopathies

• Non-transferrin-bound iron [ Time Frame: 2 hours after transfusion ] [ Designated as safety issue: Yes ]
  

• transferrin saturation [ Time Frame: 2 hours following transfusion ] [ Designated as safety issue: No ]
  
• pro-inflammatory cytokines [ Time Frame: 2 hours after transfusion ] [ Designated as safety issue: No ]
  Interleukin (IL)-6 and monocyte chemoattractant protein (MCP)-1 will be measured.
  
  

Biological: Transfusion
1 or 2 unit transfusion of fresh (defined as less than 14 days of storage) packed red blood cells stored in an additive solution vs. 1 or 2 unit transfusion of older (defined as greater than 28 days of storage) packed red blood cells stored in an additive solution.
Other Names:
• Washed red blood cells
• Cryopreserved red blood cells

Below is a summary of the procedures to be performed on each donor participant followed by the procedures for each recipient participant:

Donor participants:

Screening 2 tubes of blood will be drawn from a peripheral vein to perform a complete blood count and to perform blood bank testing (e.g. antibody screen, red cell phenotype). All donors who qualify based on criteria listed below, and have a red cell phenotype that matches a specific study patient will be recruited to perform 4 double red blood cell donations at the New York Blood Center over a 3 -year period based on the recipient's transfusion schedule. At least 112 days will be allotted between each blood donation (as per FDA regulations). Donors will have to pass the standard screening process (per FDA regulations) performed at the Blood Center, including standard infectious disease testing. Any donor failing screening due to infectious disease testing will be notified by trained counselors at the NY Blood Center and will be excluded from study participation. In addition, a new dedicated donor will replace any participant that drops out of the study, or becomes ineligible on subsequent attempts after successfully donating.

Recipient participants:

Study transfusions will be scheduled according to the patient's regular transfusion schedule such that one unit of the double red blood cell donation can be transfused "fresh" (i.e. prior to 14 days of storage) and the other unit can be transfused "old" (i.e. after 28 days of storage). The 8 transfusion events are listed below:

1. "Fresh" transfusion while patient OFF chelation therapy
2. "Old" transfusion while patient OFF chelation therapy
3. "Fresh" transfusion while patient ON chelation therapy
4. "Old" transfusion while patient ON chelation therapy
5. "Fresh" washed transfusion while patient OFF chelation therapy
6. "Old" washed transfusion while patient OFF chelation therapy
7. "Fresh" transfusion while patient OFF chelation therapy
8. Cryopreserved transfusion while patient OFF chelation therapy

For the 6 transfusions off chelation therapy, the patient will be asked to discontinue chelation therapy for 3 days prior to the scheduled transfusion. The washed and cryopreserved red cell units will be obtained per standard operating procedure from the New York Blood Center. Cryopreservation of one of the two donated double red blood cell units will be performed using Standard Operating Procedures at the New York Blood Center within 3 days of collection. Washing older stored RBC units and deglycerolizing cryopreserved red blood cell units will both be performed within 24 hours of transfusion, also by NYBC Standard Operating Procedures. Deglycerolization and washing of red blood cell units will each be performed using an automatic cell washing system (COBE 2991, CaridianBCT). All red cell units will be issued by the CUMC blood bank following standard operating procedures and a crossmatch.

The transfusion will take place in the Pediatric Hematology Clinic infusion center following standard procedures. An intravenous line will be placed and 4 tubes of blood will be drawn from the IV line prior to transfusion, immediately after transfusion, 1 hour after transfusion, and 2 hours after transfusion.