Pure white cell aplasia : Report of the first case associated with primary biliary cirrhosis

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...

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Bibliographic Details
Published in:International journal of hematology 2007-02, Vol.85 (2), p.97-100
Main Authors: TAMURA, Hideto, OKAMOTO, Muneo, YAMASHITA, Taishi, SATO, Chikako, WATANABE, Ayako, KONDO, Asaka, TATSUGUCHI, Atsushi, TSUJI, Takashi, OGATA, Kiyoyuki, DAN, Kazuo
Format: Article
Language:English
Subjects:
Aged, 80 and over
Agranulocytosis - blood
Agranulocytosis - drug therapy
Agranulocytosis - etiology
Agranulocytosis - pathology
Anti-Inflammatory Agents - administration & dosage
Biological and medical sciences
Bone Marrow - pathology
Erythropoiesis - drug effects
Fatal Outcome
Female
Gastroenterology. Liver. Pancreas. Abdomen
Hematologic and hematopoietic diseases
Humans
Infection - blood
Infection - etiology
Infection - pathology
Leukopoiesis - drug effects
Liver Cirrhosis, Biliary - blood
Liver Cirrhosis, Biliary - complications
Liver Cirrhosis, Biliary - drug therapy
Liver Cirrhosis, Biliary - pathology
Liver. Biliary tract. Portal circulation. Exocrine pancreas
Medical sciences
Methylprednisolone - administration & dosage
Other diseases. Hematologic involvement in other diseases
Other diseases. Semiology
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Summary: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.
ISSN:0925-5710
1865-3774
DOI:10.1532/IJH97.06018