Abstract
A 21 month old female was admitted to hospital because of recurrent skin and upper respiratory infections and chronic neutropenia of 3 months duration. Family history was unremarkable and past history was negative for any known causes of neutropenia. Physical examination revealed multiple skin abscesses and marked hepatosplenomegaly. Absolute granulocyte count varied between 150 and 500/μl; segmented neutrophils were absent in the bone marrow. RBC and platelet count were normal.
Immunological evaluation revealed normal C3, C4 and total hemolytic complement. Serum immunoglobulins demonstrated absent IgA, low IgG (170 and 120 mg/dl) and elevated IgM (190 and 740 mg/dl) at 21 and 27 months, respectively. Isohemagglutinins were absent (blood type O, Rh positive). Despite previous immunization, polio antibody titers were <1:4 for all types, but an antibody response followed antigenic challenge with diphtheriatetanus antigens. There was no response to skin tests with SKSD and monilial antigens. E-rosette forming lymphocytes were diminished (36% vs 60% control) and PHA-stimulation was minimal (1 to 2-fold vs 11 to 24). Staining for immunoglobulin bearing B-cells was variable: IgG-15% and 51%, IgA-13% and 1%, IgM-7% and 8%, at 21 and 27 months, respectively. Serum leukoagglutinins were present with 5 panels of control cells.
This case represents the first report of neutropenia with B-and T-cell deficiency and circulating leukoagglutinins.
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Schulman, J., Mutz, I. & Shearer, W. CHRONIC NEUTROPENIA WITH LEUKOAGGLUTININS AND B- AND T-CELL IMMUNODEFICIENCY. Pediatr Res 11, 493 (1977). https://doi.org/10.1203/00006450-197704000-00742
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DOI: https://doi.org/10.1203/00006450-197704000-00742
