Neutropenie door antistoffen tegen Fc-receptoren type III op neutrofiele granulocyten: 3 kinderen met verschillend ziektebeloop

Three children (girls) suffered from neutropenia mediated by anti-neutrophil IgG-Fc receptor type III (FcγRIII) antibodies. The first patient (newborn) had asymptomatic and transient neutropenia caused by maternal FcγRIII iso-antibodies. The second patient (6 months), whose neutropenia was diagnosed as a ‘benign neutropenia of childhood’ caused by transient anti-NA1 autoantibodies, suffered from mild bacterial infections. The third patient (12 years) suffered from serious infections. The anti-FcγRIII autoantibodies showed neither anti-NA1 nor anti-NA2 specificity. She also developed autoimmune thyroiditis (Graves' disease). Both the duration of the neutropenia and the seriousness of the bacterial infection were variable in our patient group. The first two patients both made spontaneous recoveries, while the third patient depended ultimately on granulocyte-colony stimulating factor (G-CSF).