Abstract
A sixteen year old white male with X-linked Type I Dysgammaglobulinemia developed persistent neutropenia (total neutrophils < 500/mm3) which was unresponsive to broad spectrum antibiotics, prednisone, or a single infusion of one liter of fresh frozen plasma. His plasma agglutinated neutrophils in a microagglutination assay. His plasma (or serum) inhibited colony formation in a dose dependent fashion in a granulocyte/macrophage colony forming assay using human bone marrow cells. Complete inhibition was obtained when the patient's serum reached a final concentration of 5%. This effect was similar when either the patient's or normal bone marrow cells were used. Heating the patient's serum to 56°C for 30 minutes did not destroy the inhibitor. Bone marrow cells cultured in a medium containing 5% of the patient's serum and 5% of normal serum were also completely inhibited. Seven total plasma exchange procedures were performed over three weeks during which time the peripheral neutrophil count returned to normal and no serum inhibitor could be demonstrated. Upon completion of the plasma exchange procedures, the peripheral neutrophil count fell within three days to less than 500/mm3 and the serum inhibitor of colony formation reappeared. Three weeks after the plasma exchange procedures, the neutrophil count spontaneously returned to normal while on broad spectrum antibiotic therapy.
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