A variant of myelokathexis with hypogammaglobulinemia: Lymphocytes as wellas neutrophils may reverse in response to infections

A 7-year-old boy with prolonged and marked leukopenia diagnosed at 6 months of age is described. The polymorphonuclear cells presented no hypersegment ed nuclei or concentrated nuclear chromatin, although vacuolated myeloid ce lls appeared in bone marrow smears. Neutrophils reversed in response to adm inistration of G-CSF. His leukocyte counts were 400-1000/mu L during afebri le periods and increased to 2000-3000/mu L in response to infections. The i ncreased leukocyte was usually neutrophils, but lymphocytes also increased at EB-virus infection. The serum IgG decreased gradually and was 364 mg/dL at 7 years of age. Antibody responses were normal and recurrent otitis medi a has been the patient's only problem. Granulocytopenia with hypogammaglobu linemia of this patient mimics myelokathexis with hypogammaglobulinemia, an d lymphocytes also increased at viral infections.