title: Pure white cell aplasia: report of the first case associated with primary biliary cirrhosis.
abstract: Pure white cell aplasia (PWCA) is a rare hematologic disorder characterized by agranulocytosis, a lack of virtually all neutrophil-lineage cells (from neutrophils to myeloblasts) in the bone marrow, and normal erythropoiesis and megakaryocy-topoiesis. We report the first case of PWCA that developed in a patient with primary biliary cirrhosis (PBC). An 83-year-old woman, who had had an elevated serum alkaline phosphatase level and shown positivity for serum antimitochondrial antibodies for 10 years, was referred to us because of a perianal abscess. She had severe neutropenia, and her bone marrow showed typical findings of PWCA. Although methylprednisolone pulse therapy induced complete neutrophil recovery, this effect was transient. She died of infection, and the autopsy confirmed the diagnosis of PBC. In vitro investigations showed that factors inhibitory to normal CD34 cell-derived granulopoiesis were present in the patient's serum.
authors.affiliation: Division of Hematology, Department of Medicine, Nippon Medical School, Tokyo, Japan.
authors.forename: Hideto
authors.initials: H
authors.lastname: Tamura
authors.forename: Muneo
authors.initials: M
authors.lastname: Okamoto
authors.forename: Taishi
authors.initials: T
authors.lastname: Yamashita
authors.forename: Chikako
authors.initials: C
authors.lastname: Sato
authors.forename: Ayako
authors.initials: A
authors.lastname: Watanabe
authors.forename: Asaka
authors.initials: A
authors.lastname: Kondo
authors.forename: Atsushi
authors.initials: A
authors.lastname: Tatsuguchi
authors.forename: Takashi
authors.initials: T
authors.lastname: Tsuji
authors.forename: Kiyoyuki
authors.initials: K
authors.lastname: Ogata
authors.forename: Kazuo
authors.initials: K
authors.lastname: Dan
mesh_terms: D000369:Aged, 80 and over; D000380:Agranulocytosis / Q000097:blood / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D001853:Bone Marrow / Q000473:pathology; D004920:Erythropoiesis / Q000187:drug effects; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007239:Infections / Q000097:blood / Q000209:etiology / Q000473:pathology; D019891:Leukopoiesis / Q000187:drug effects; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D008775:Methylprednisolone / Q000008:administration & dosage
chemical_list: D000893:Anti-Inflammatory Agents; D008775:Methylprednisolone