Severe acquired neutropenia associated with anti-proteinase 3 antibodies.

摘要

Anti-neutrophil cytoplasmic antibodies (ANCA) are associated with a large spectrum of diseases, including Wegener's disease, microscopic polyarteritis and necro-tizing crescentic glomerulonephritis. The responsibility of ANCA in the pathogenesis of chronic neutropenia has recently been suggested by the description of 9 cases of severe neutropenia associated with the presence of ANCA with various antigenic specificities. Here, we report a new case of severe acquired chronic neutropenia associated with the detection of both ANCA and anti-surface neu-trophil antibodies and discuss the relationship with this immune disease.