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Granulocyte transfusion therapy in a child with chronic granulomatous disease and multiple red cell alloantibodies
L Depalma1, S F Leitman, C S Carter
1Department of Transfusion Medicine, National Institute of Allergy and Infectious Diseases, Bethesda, Maryland.
Insights
A novel method efficiently reduces red blood cells (RBCs) in granulocyte concentrates, enabling safe daily transfusions for patients with RBC alloimmunization. This supports treatment for conditions like chronic granulomatous disease.
Area of Science:
- Hematology
- Immunology
- Transfusion Medicine
Background:
- Patients with chronic granulomatous disease (CGD) may develop red cell (RBC) alloantibodies, complicating treatment.
- Standard antibiotic therapy may be insufficient for severe infections in CGD patients, necessitating alternative treatments like granulocyte transfusions.
Observation:
- A pediatric patient with CGD, nocardial pneumonia, and osteomyelitis developed multiple RBC alloantibodies (anti-c, -E, -Jka).
- A method involving leukapheresis, gravity sedimentation, and centrifugation was developed to reduce incompatible RBCs in granulocyte concentrates.
Findings:
- The described method significantly reduced RBC content in granulocyte concentrates (from 29 +/- 7 to 2.5 +/- 1.0 ml) while maintaining high white cell recovery (70%).
- Daily granulocyte transfusions using this method were safely administered to the patient, even with mismatched RBCs, without clinical or laboratory signs of hemolysis.
- The processed product consistently contained a therapeutic dose of granulocytes (1.6 +/- 1.0 X 10(10)).
Implications:
- This technique offers a viable solution for providing daily granulocyte therapy to alloimmunized patients when phenotypically matched donors are unavailable.
- The method's simplicity and efficiency may improve treatment accessibility for patients requiring granulocyte support, particularly those with complex transfusion histories.
- This approach could enhance the management of severe infections in immunocompromised individuals, including those with CGD and other hematologic disorders.
Abstract:
A 7-year-old, 17-kg child with chronic granulomatous disease and nocardial pneumonia and osteomyelitis did not respond to antibiotic therapy and developed multiple red cell (RBC) alloantibodies (anti-c, -E, and -Jka). To provide daily granulocyte concentrates, a method was devised to reduce the number of incompatible RBCs per transfusion. Leukapheresis was done with hydroxyethyl starch, and the apheresis product was allowed to sediment by gravity in a plasma expressor for 90 minutes. The leukocyte-rich plasma was separated from the sedimented RBCs by transfer to a satellite bag, and the volume of the product was reduced by centrifugation to approximately 80 ml. RBC content was reduced from 29 +/- 7 to 2.5 +/- 1.0 ml (n = 22, p less than 0.01) and was accompanied by a 70 percent recovery of white cells (range, 49-90%). The final product contained 1.6 +/- 1.0 X 10(10) granulocytes. There were no clinical or laboratory signs of hemolysis during the course of 46 granulocyte transfusions, 37 of which were derived from c-, E-, or Jka-positive donors. The size of most apheresis donor pools is insufficient to provide phenotypically matched granulocyte concentrates daily for patients with RBC alloimmunization. The rapid, simple method described here may allow daily therapy with mismatched concentrates to be administered safely to such patients.