Clonal T cell-mediated cyclic thrombocytopenia.
Cyclic thrombocytopenia is a rare disorder characterized by periodic platelet count fluctuations of unknown aetiology. We report on a female patient with cyclic changes of platelet counts ranging from 6 x 10(9)/l to 753 x 10(9)/l in 4-week intervals. Platelet counts were inversely correlated to thrombopoietin levels suggesting production failure. Reticulocyte counts and neutrophil counts showed similar, but less prominent, fluctuations. Clonal T-cell receptor rearrangement was detected in bone marrow samples as well as in peripheral blood. Cell typing of blood lymphocytes revealed a relative increase in CD3+ T cells. Treatment with cyclosporine A resulted in a substantial improvement of platelet counts. Taken together, we provide evidence for clonal T-cell mediated bone marrow failure with cyclic impairment of thrombopoiesis responsive to cyclosporine therapy.
Füreder
Wolfgang
W
Department of Internal Medicine I, Division of Haematology & Haemostaseology, The University of Vienna, Austria. vofuer@netway.at
Mitterbauer
Gerlinde
G
Thalhammer
Renate
R
Geissler
Klaus
K
Panzer
Simon
S
Krebs
Michael
M
Simonitsch-Klupp
Ingrig
I
Sperr
Wolfgang R
WR
Lechner
Klaus
K
Kyrle
Paul A
PA
D016572:Cyclosporine / Q000627:therapeutic use; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte; D006801:Humans; D016130:Immunophenotyping; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007958:Leukocyte Count; D008875:Middle Aged; D010507:Periodicity; D010976:Platelet Count; D016176:T-Lymphocyte Subsets / Q000276:immunology*; D013921:Thrombocytopenia / Q000097:blood / Q000188:drug therapy / Q000276:immunology*; D013926:Thrombopoietin / Q000097:blood
D007166:Immunosuppressive Agents; D016572:Cyclosporine; D013926:Thrombopoietin