Survival of transfused normal granulocytes in a patient with chronic granulomatous disease.

A 5-year-old boy with chronic granulomatous disease (CGD) received four granulocyte transfusions from unrelated HLA-matched donors as part of therapy for a hepatic abscess. Survival studies of transfused granulocytes using the endotoxin-stimulated nitroblue tetrazolium (NBT) test on two occasions demonstrated 19.6% and 16.8% transfusion efficiency and a biphasic granulocyte disappearance curve. These curves were similar whether or not the patient had serum leukoagglutinins directed against donor granulocytes. Transfused granulocytes were present 24 hours after transfusion. Parallel studies demonstrated normal stimulated NBT activity of donor PMNs after overnight storage at 4 C. Kinetic studies of transfused granulocytes in the non-neutropenic recipient with CGD may be performed without radioactive labeling of granulocytes because of the distinctive metabolic abnormality of their cells compared with normal donor granulocytes.