Severe autoimmune neutropenia associated with acute autoimmune hepatitis.
A 49-year-old previously healthy female presented with acute hepatitis and severe neutropenia. A diagnosis of type 1 autoimmune hepatitis was made based on the histological appearance of a liver core biopsy, positive anti-smooth muscle antibodies, and positive anti-neutrophil cytoplasmic antibody (atypical ANCA). Hemogram revealed mild leukopenia with severe neutropenia (absolute neutrophil count 256/mm(3)), normal hemoglobin and mild thrombocytopenia (115000/mm(3)). A bone marrow biopsy and aspirate had a normal karyotype, increase in granulopoiesis, prominence of promyelocytes (31%) and absence of mature granulocytes. Anti-neutrophil antibodies were detected in the patient's blood. Therapy was directed at the underlying hepatitis with resolution of neutropenia without the use of colony-stimulating factors.
Wehbe
Ahmad M
AM
Division of Hematology, Oncology and Blood and Marrow Transplantation, Department of Internal Medicine, University of Iowa Hospitals and Clinics, Iowa City, IA 52246, USA. ahmad-wehbe@uiowa.edu
Johannsson
Birgir
B
Raife
Thomas J
TJ
Bleile
Michelle
M
Bell
Adam
A
Curtis
Brian R
BR
Halfdanarson
Thorvardur R
TR
D001323:Autoantibodies / Q000097:blood; D001706:Biopsy; D005260:Female; D019693:Hepatitis, Autoimmune / Q000150:complications* / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007958:Leukocyte Count; D008099:Liver / Q000473:pathology; D008875:Middle Aged; D009503:Neutropenia / Q000209:etiology* / Q000276:immunology* / Q000473:pathology; D012720:Severity of Illness Index
D001323:Autoantibodies