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 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.