2-Deoxycoformycin in the treatment of T-large granular lymphocyte leukemia
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- 作者：Tsirigotis ; Panagiotis ; Venetis ; Evangelos ; Kapsimali ; Violeta ; Rontogianni ; Dimitra ; Varvitsioti ; Ephrosini ; et. al.
- 关键词：T-LGL ; Neutropenia ; 2-Deoxycoformycin ; Pentostatin ; Vβ ; repertoire
- 刊名：Leukemia Research
- 出版年：2003
- 出版时间：September, 2003
- 年：2003
- 卷：27
- 期：9
- 页码：865-867
- 全文大小：42.7 K
文摘
A 52-year-old woman presented to our clinic for investigation of agranulocytosis and mild lymphocytosis. A diagnosis of T-large granular lymphocyte leukemia was made, based on immunophenotyping findings of the peripheral blood lymphocytes (CD3, CD8, CD16, CD57). Flow cytometric analysis of TCR-Vβ repertoire showed single Vβ9 expression on peripheral T-cells. Clonality was also demonstrated with PCR analysis which revealed clonal rearrangement of TCRγ-chain gene. Granulocyte-macrophage colony-stimulating factor (G-CSF) (G-CSF), cyclosporine, methylprednisolone and oral methotrexate failed to correct the neutropenia. Finally, treatment with 2-deoxycoformycin (DCF) was successful and resulted in complete correction of the neutrophil count. Flow cytometric analysis of TCR-Vβ repertoire proved to be an effective method to assess the therapeutic response to various treatments and to evaluate residual disease.