Multiple autoimmune haemopoietic disorders and insidious clonal proliferation of large granular lymphocytes.
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.
Akashi
K
K
First Department of Internal Medicine, Kyushu University, Fukuoka, Japan. akashi@leland.stanford.edu
Shibuya
T
T
Taniguchi
S
S
Hayashi
S
S
Iwasaki
H
H
Teshima
T
T
Takamatsu
Y
Y
Gondo
H
H
Okamura
T
T
Harada
M
M
Niho
Y
Y
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