細胞傷害性抗好中球抗体を証明しえたImmune-Neutropeniaの1例

抄録

A case of immune-neutropenia with anti-neutrophil-antibody, which had cytotoxic activities on normal neutrophils, is reprted.
A 34-year-old woman was admitted to our hospital because of chronic neutropenia. She had never experienced recurrent bacterial infections after developing neutropenia. Hematological examination on admission showed leukopenia (1,100/cmm) and severe neutropenia (8%). Bone marrow aspiration revealed marked decrease of segmented neutrophils. Anti-nuclear-antibody, direct Coombs' test, cold agglutinin, anti-thyroglobulin and microsome antibodies were positive. Other auto-antibodies, circulating immune-copmplex and anti-HLA antibodies were not detected. Mild hepato-splenomegaly was revealed by gallium scintigraphy and abdominal CT. This antineutrophil-antibody had cytotoxic activities on normal peripheral neutrophils, which was demonstrated by ⁵¹Cr release assay with complement-mediated cytotoxic reaction. The patient had not any other clinical and laboratory signs for SLE, and so a diagnosis of immune (probably autoimmune) neutropenia was made from these findings. Although her neutropenia was not improved by steroid (including steroid pulse therapy), azatiopurine nor high-dose immunoglobulin application, it was improved mildly after splenectomy.