Neutropenia associated with X-linked agammaglobulinaemia

In a series of six cases of sex-linked agammaglobulinaemia neutropenia occurred as a presenting feature in four and during the presenting illness in the other two. The six patients all had low antibody titres and absent or low immunoglobulin concentrations with normal concentrations of T cells and absent B cells. The patients were all first seen with severe, acute infection, including septic abscesses and meningitis; neutropenia resolved as the infection and immunoglobulin deficiency were treated. Haematologists should be aware that neutropenia is a common association of infection in patients with immunoglobulin deficiency.