演題詳細

ポスター / Poster

ポスター 14 (Poster 14) :MPN:臨床 1

print

日程
2013年10月11日(金)
時間
16:50 - 17:50
会場
ポスター会場 / Poster (ロイトン札幌 3F ロイトンホールABCD)
座長・司会
藤 重夫 (Shigeo Fuji):1
1:国立がん研究センター中央病院 移植科
 
前へ戻る

A t(8;22) myeloproliferative neoplasm transforming to lymphoid blast crisis: A case report

演題番号 : PS-1-101

藤本 哲広 (Tetsuhiro Fujimoto):1、太良 史郎 (Shiro Tara):1、下村 泰三 (Taizo Shimomura):1、鈴島 仁 (Hitoshi Suzushima):1

1:Department of Hematology, Kumamoto Shinto General Hospital

 

Hematopoietic myeloproliferative neoplasms (MPNs) with rearrangements of the receptor tyrosine kinase fibroblast growth factor1 (FGFR1) gene, located on chromosome 8p11, are uncommon. Involvement of t(8;22)(p11;q11)/BCR-FGFR1 is exceedingly rare with only 10 cases reported to date. We herein report a patient with a clinical and hematologic diagnosis of chronic myeloid leukemia (CML) in chronic phase who had an acquired t(8;22)(p11;q11). Analysis by fluorescence in situ hybridization (FISH) indicated that the patient was negative for the BCR-ABL fusion. Further analysis by FISH indicated a breakpoint within FGFR1. The patient was initially treated with 50mg/day of dasatinib, which failed to decrease the white cells bearing the t(8;22) translocation and to induce hematologic remission. Therefore, the patient subsequently received 1,000mg/day of hydroxyurea as cytoreduction therapy, which successfully resulted in the decrease in the total white cell count. Twelve months later, however, the patient developed lymphoid blast crisis with additional chromosomal abnormalities. The patient received chemotherapy with vincristine, prednisone and daunorubicin, which reduced the cellularity and rapidly progressed to a myeloablative, unrelated donor hematopoietic stem cell transplantation. Nevertheless, the patient showed disease progression and died. This is the first description of a patient with a t(8;22) MPN presenting lymphoid blast crisis.

前へ戻る