Chronic granulomatous disease and the Mcleod phenotype. Successful treatment of infection with granulocyte transfusions resulting in subsequent hemolytic transfusion reaction.
A young man with X-linked chronic granulomatous disease of childhood, who is of the rare McLeod phenotype with antibodies in his serum shown to be hemolytic and reactive against all red cells with normal expressions of the Kell antigens, developed a severe Nocardia pneumonia with abscess formation and was subsequently treated successfully with granulocyte transfusions in spite of the presence of anti-KX in the patient's serum. The anti-KX did not appear to alter significantly the effectiveness of the transfused granulocytes; it did, however, cause a mild hemolytic transfusion reaction. The patient made a remarkable recovery from this episode and his condition has progressed to a state satisfactory enough for him to donate his own blood for storage and possible use in the future.
D000328:Adult; D001789:Blood Group Antigens; D001787:Blood Group Incompatibility / Q000209:etiology*; D001803:Blood Transfusion; D006098:Granulocytes / Q000637:transplantation*; D006105:Granulomatous Disease, Chronic / Q000097:blood* / Q000150:complications* / Q000235:genetics; D006801:Humans; D007626:Kell Blood-Group System; D017708:Leukocyte Transfusion; D008297:Male; D009617:Nocardia Infections / Q000150:complications / Q000628:therapy; D010375:Pedigree; D010585:Phagocyte Bactericidal Dysfunction / Q000097:blood* / Q000150:complications*; D010641:Phenotype; D011014:Pneumonia / Q000150:complications / Q000628:therapy
D001789:Blood Group Antigens; D007626:Kell Blood-Group System