Autoimmune neutropenia due to antineutrophil antibodies in a patient with primary sclerosing cholangitis

Abstract

Autoimmune neutropenia (AIN) is defined as a decrease in the circulating absolute neutrophil count (ANC) to less than 1500/μl caused by serum antineutrophil antibodies. Secondary AIN is associated with various autoimmune diseases. Herein we present the case of a patient with primary sclerosing cholangitis (PSC) who developed secondary AIN. A 19-year-old man was admitted due to liver injury, and a diagnosis of PSC was established by cholangiogram and liver biopsy. Severe neutropenia, with the ANC down to 130/μl, developed during his hospital course. No medications had been given at that time and bone marrow aspiration revealed no abnormality. Therefore we suspected secondary AIN as a causative etiology and examined whether antineutrophil antibodies were detectable in the patient's sera by flow cytometric analysis of the granulocyte indirect immunofluorescence test. We found that antineutrophil antibody was strongly positive on admission, and the titer decreased along with recovery from neutropenia. This is the first reported case of a patient with PSC who developed AIN, with detection of serum antineutrophil antibodies.

Antineutrophil Cytoplasmic Antibodies, Autoimmune Neutropenia, and Vasculitis

“…Autoimmune liver diseases, including autoimmune hepatitis and primary sclerosis cholangitis, have been associated with non-specific ANCA, presence of antineutrophil membrane antibodies, and severe neutropenia in the absence of vasculitis (24–26). ANCA and severe neutropenia has also been described in three cases of Sjögren's syndrome, and in one case, cutaneous vasculitis and antineutrophil membrane antibodies were reported (27–28).…”