title: Autoimmune-associated hemophagocytic syndrome originating from autoimmune hepatitis with a successful response to therapy.
abstract: The patient was a 15-year-old girl with severe acute hepatitis. A liver biopsy showed the typical findings of autoimmune hepatitis (AIH). Subsequently, two lineages of cytopenia were found in the patient's peripheral blood. Hemophagocytosis by macrophages was observed in the bone marrow. Virus-, drug- and lymphoma-associated hemophagocytic syndrome (HPS) was ruled out. Therefore, the patient was diagnosed with autoimmune-associated HPS (AAHS). Following the administration of combination therapy with prednisolone and cyclosporine A, both the AAHS and AIH improved. This is the first report of AAHS originating from AIH. The patient was followed up for five years after treatment, and no disease recurrence was detected.
authors.affiliation: Division of Gastroenterology, Department of Internal Medicine, Kobe University Graduate School of Medicine, Japan.
authors.forename: Masaya
authors.initials: M
authors.lastname: Saito
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Yano
authors.forename: Akihiro
authors.initials: A
authors.lastname: Minami
authors.forename: Kenji
authors.initials: K
authors.lastname: Momose
authors.forename: Hirotaka
authors.initials: H
authors.lastname: Hirano
authors.forename: Takashi
authors.initials: T
authors.lastname: Yamasaki
authors.forename: Tomoo
authors.initials: T
authors.lastname: Itoh
authors.forename: Masaru
authors.initials: M
authors.lastname: Yoshida
authors.forename: Takeshi
authors.initials: T
authors.lastname: Azuma
mesh_terms: D000293:Adolescent; D044963:Biopsy, Fine-Needle; D001772:Blood Cell Count; D001853:Bone Marrow / Q000473:pathology; D019070:Cell Lineage; D016572:Cyclosporine / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D019693:Hepatitis, Autoimmune / Q000097:blood / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D008099:Liver / Q000473:pathology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood / Q000188:drug therapy / Q000209:etiology*; D011239:Prednisolone / Q000627:therapeutic use; D012074:Remission Induction
chemical_list: D016572:Cyclosporine; D011239:Prednisolone