title: 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.
authors.affiliation: Department of Medicine, Teikyo University School of Medicine, 2-11-1 Kaga, Itabashi-ku, Tokyo, 173-8605, Japan.
authors.forename: Naoko
authors.initials: N
authors.lastname: Hanawa
authors.affiliation: Department of Medicine, Teikyo University School of Medicine, 2-11-1 Kaga, Itabashi-ku, Tokyo, 173-8605, Japan. a-tanaka@med.teikyo-u.ac.jp.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Tanaka
authors.affiliation: Department of Medicine, Teikyo University School of Medicine, 2-11-1 Kaga, Itabashi-ku, Tokyo, 173-8605, Japan.
authors.forename: Masako
authors.initials: M
authors.lastname: Fukami
authors.affiliation: Department of Medicine, Teikyo University School of Medicine, 2-11-1 Kaga, Itabashi-ku, Tokyo, 173-8605, Japan.
authors.forename: Ryo
authors.initials: R
authors.lastname: Miura
authors.affiliation: Department of Medicine, Teikyo University School of Medicine, 2-11-1 Kaga, Itabashi-ku, Tokyo, 173-8605, Japan.
authors.forename: Hideaki
authors.initials: H
authors.lastname: Goto
authors.affiliation: Department of Medicine, Teikyo University School of Medicine, 2-11-1 Kaga, Itabashi-ku, Tokyo, 173-8605, Japan.
authors.forename: Haruko
authors.initials: H
authors.lastname: Tashiro
authors.affiliation: Department of Medicine, Teikyo University School of Medicine, 2-11-1 Kaga, Itabashi-ku, Tokyo, 173-8605, Japan.
authors.forename: Mitsuhiko
authors.initials: M
authors.lastname: Aiso
authors.affiliation: Department of Medicine, Teikyo University School of Medicine, 2-11-1 Kaga, Itabashi-ku, Tokyo, 173-8605, Japan.
authors.forename: Yoriyuki
authors.initials: Y
authors.lastname: Takamori
authors.affiliation: Department of Internal Medicine, St. Luke's International Hospital, Tokyo, 104-8560, Japan.
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Fujita
authors.affiliation: Department of Pediatrics, Graduate School of Biomedical Sciences, Hiroshima University, Hiroshima, 734-8551, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Sato
authors.affiliation: Department of Pediatrics, Graduate School of Biomedical Sciences, Hiroshima University, Hiroshima, 734-8551, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kawaguchi
authors.affiliation: Department of Pediatrics, Graduate School of Biomedical Sciences, Hiroshima University, Hiroshima, 734-8551, Japan.
authors.forename: Masao
authors.initials: M
authors.lastname: Kobayashi
authors.affiliation: Department of Medicine, Teikyo University School of Medicine, 2-11-1 Kaga, Itabashi-ku, Tokyo, 173-8605, Japan.
authors.forename: Hajime
authors.initials: H
authors.lastname: Takikawa
keywords: Autoimmune disease; Cholestasis; Granulocyte indirect immunofluorescence test; Neutropenia