Prolonged neutropenia resulting from antibodies to neutrophil-specific antigen NB1 following marrow transplantation.
Marrow graft failure is a significant cause of morbidity following bone marrow transplantation. A case is reported of marrow graft failure due to neutrophil antibodies. A 13-year-old girl with a large granular lymphocytosis and chronic neutropenia was treated with granulocyte transfusions prior to undergoing a transplant with bone marrow from a partially matched, unrelated donor. Following the transplant, a bone marrow biopsy showed engraftment of donor myeloid cells, but the recipient remained neutropenic. Testing of the serum for neutrophil antibodies found that the recipient's serum had a high-titer neutrophil antibody. Immunoprecipitation studies using the marrow recipient's serum and 125I surface-labeled neutrophils showed that the antibody reacted to the neutrophil-specific antigen NB1. Phenotyping of neutrophils from the marrow donor found that they expressed NB1 antigen, and, in a crossmatch assay, the recipient's serum reacted with donor neutrophils. Despite treatment with granulocyte-macrophage--colony-stimulating factor, the marrow transplant recipient remained neutropenic and died of polymicrobial sepsis and aspergillosis 38 days after the transplant. The presence of high-titer antibodies to neutrophil-specific antigen NB1 in this patient following transplant likely prevented the recovery of her peripheral blood neutrophils and contributed to her death.
Stroncek
D F
DF
Department of Laboratory Medicine, University of Minnesota Medical School, Minneapolis.
Shapiro
R S
RS
Filipovich
A H
AH
Plachta
L B
LB
Clay
M E
ME
D000293:Adolescent; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D005260:Female; D058851:GPI-Linked Proteins; D006801:Humans; D007518:Isoantibodies / Q000009:adverse effects* / Q000097:blood; D007519:Isoantigens / Q000235:genetics / Q000276:immunology*; D008562:Membrane Glycoproteins / Q000235:genetics / Q000276:immunology*; D009503:Neutropenia / Q000209:etiology* / Q000276:immunology*; D009504:Neutrophils / Q000276:immunology; D010641:Phenotype; D011956:Receptors, Cell Surface
C407242:CD177 protein, human; D058851:GPI-Linked Proteins; D007518:Isoantibodies; D007519:Isoantigens; D008562:Membrane Glycoproteins; D011956:Receptors, Cell Surface