title: Multiple autoimmune haemopoietic disorders and insidious clonal proliferation of large granular lymphocytes.
abstract: We report a patient with clonal proliferation of CD3+8+TCRalphabeta+ large granular lymphocytes (LGL) presenting multiple episodes of autoimmune cytopenia, including autoimmune neutropenia, idiopathic thrombocytopenic purpura, autoimmune haemolytic anaemia, and pure red cell aplasia. Each disorder appeared separately or as a combination during an 11-year clinical course. The increase of blood CD3+8+TCRalphabeta+ LGL was detected 6 years after the initial diagnosis of cytopenia, but the absolute number of LGL cells was always < 1.0 x 109/l. LGL cells were of monoclonal origin and had a chromosomal abnormality. LGL cells transiently responded to cyclosporine A therapy, which was also effective on all of these autoimmune cytopenias. Accordingly, an undetectable level of proliferation of a clonal LGL population could cause various autoimmune haemopoietic disorders.
authors.affiliation: First Department of Internal Medicine, Kyushu University, Fukuoka, Japan. akashi@leland.stanford.edu
authors.forename: K
authors.initials: K
authors.lastname: Akashi
authors.forename: T
authors.initials: T
authors.lastname: Shibuya
authors.forename: S
authors.initials: S
authors.lastname: Taniguchi
authors.forename: S
authors.initials: S
authors.lastname: Hayashi
authors.forename: H
authors.initials: H
authors.lastname: Iwasaki
authors.forename: T
authors.initials: T
authors.lastname: Teshima
authors.forename: Y
authors.initials: Y
authors.lastname: Takamatsu
authors.forename: H
authors.initials: H
authors.lastname: Gondo
authors.forename: T
authors.initials: T
authors.lastname: Okamura
authors.forename: M
authors.initials: M
authors.lastname: Harada
authors.forename: Y
authors.initials: Y
authors.lastname: Niho
mesh_terms: D000293:Adolescent; D000743:Anemia, Hemolytic / Q000209:etiology / Q000473:pathology; D001327:Autoimmune Diseases / Q000473:pathology*; D001772:Blood Cell Count; D002455:Cell Division; D005260:Female; D006801:Humans; D007945:Leukemia, Lymphoid / Q000473:pathology*; D009503:Neutropenia / Q000473:pathology*; D013921:Thrombocytopenia / Q000209:etiology / Q000473:pathology