Clinical and Immunophenotypic Properties of Small Cell Variant of T-cell Prolymphocytic Leukemia / 中国实验血液学杂志

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the clinical, morphologic and immunophenotypic properties of the patients with small cell variant of T-cell prolymphocytic leukaemia(T-PLL).</p><p><b>METHODS</b>Peripheral blood and bone marrow cytomorphologic and immunophenotypic examination, and T-cell receptor(TCR) gene rearrangement detection were used to verify the diagnosis for 2 patients with lymphocytosis. Two patients were treated with combined chemotherapeutic protocol based on fludarabine.</p><p><b>RESULTS</b>At diagnosis of case 1, the main lymphocytes of peripheral blood smear were the small mature lymphocytes without nucleoli. The immunophenotype of the cells was CD3CD5CD7CD4CD8TCRα/β. The patient achieved complete remission after treatment with combined with CTX of fludarabine. The disease relapsed at 32 months after diagnosis. The abnormal lymphocytes were medium-sized ones with a visible nucleolus. Immunophenotyping showed that the leukemic cells were predominantly CD8 positive(CD3CD5CD7CD4CD8TCRα/β). Both the peripheral blood and bone marrow cells of case 2 were predominanthy the mature lymphocytes, and their immunophenotype was HLA-DRCD7CD5CD4CD3CD2CD56cCD3TCRα/β. The combined fludarabine therapy was ineffective.</p><p><b>CONCLUSION</b>Immunophenotypical switch from CD4CD8to CD4CD8may be associated with a poor response to chemotherapy. CD56 expression is an independent poor prognostic factor for primary refractory disease in T-PLL and may be considered for implementing risked-adapted therapeutic strategies.</p>