Idiopathic thrombocytopenic purpura: Better therapeutic responses of patients with B- or T-Cell clonality than patients without clonality

Results of recent studies of the pathogenesis of idiopathic thrombocytopenic purpura (ITP) have suggested activated helper T-cells drive B-lymphocytes to produce antibodies. Twenty-eight children and 85 adults with ITP entered this study. We performed polymerase chain reaction (PCR) using framework III variable region (V-H FRIII)- and joining region (J(H))-specific primers to analyze immunoglobulin heavy-chain gene rearrangement (IgH GR) for B-cell clonality. We used multiplex PCR to analyze T-cell receptor (TCR) gamma-chain gene rearrangement (TCRgamma GR) for T-cell clonality. We diagnosed 10 cases as acute ITP and 97 cases as chronic ITP. The IgH GR result was positive in 77.8% of the acute-form cases and in 58.8% of the chronic-form cases. The TCRgamma GR result was positive in 11.1% of the acute cases and in 10.6% of the chronic cases. There was no difference in frequency of clonality between the acute and chronic forms. After treatment the platelet count normalized in 81.8% (36/44) of the chronic ITP cases with B-cell clonality and in 88.9% (8/9) of the chronic ITP cases with T-cell clonality, compared with a normalized platelet count in 46.2% (12/26) of the chronic ITP cases without clonality. The patients with T- or B-cell clonality appeared to have better therapeutic responses than patients without clonality. In conclusion, T- and B-cell clonality may play a positive role in determining therapeutic response. Int J Hematol. 2003;78:461-466. (C) 2003 The Japanese Society of Hematology.