Clinical Features and Treatment Outcomes of 81 Patients with Aggressive Type Adult T-cell Leukemia-lymphoma at a Single Institution over a 7-year Period (2006-2012)

作者：Kawano Noriaki^*; Yoshida Shuro; Kuriyama Takuro; Tahara Yoshihiro; Yamashita Kiyoshi; Nagahiro Yuri; Kawano Jiro; Koketsu Hideki; Toyofuku Atsushi; Manabe Tatsuya; Beppu Kiichiro; Ono Nobuyuki; Himeji Daisuke; Yokota Ikeda Naoko; Inoue Sanshiro; Ochiai Hidenobu; Sonoda Koh Hei; Shimoda Kazuya; Ishikawa Fumihiko; Ueda Akira

来源：Internal Medicine, 2015, 54(12): 1489-1498.

DOI：10.2169/internalmedicine.54.1953

• adult T-cell leukemia-lymphoma
• poor prognosis
• a high proportion of palliative therapy
• a small proportion of long-term survivors with chemotherapy
• a small proportion of hematopoietic stem cell transplantation

Objective Despite the remarkable advances in chemotherapy and allogeneic hematopoietic stem cell transplantation (HSCT), adult T-cell leukemia-lymphoma (ATL) is still associated with a high mortality rate. It is therefore essential to elucidate the current features of ATL.
Methods We retrospectively analyzed 81 patients with aggressive type ATL at our institution over a 7-year period based on Shimoyama's diagnostic criteria.
Results Eighty-one patients with a median age of 67.5 years were classified as having acute (n=47), lymphoma (n=32), or chronic type (n=2) ATL. They were initially treated by either palliative therapy (n=25) or systemic chemotherapy [n=56; cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) therapy (n=25)/vincristine, cyclophosphamide, doxorubicin, and prednisone (VCAP)-doxorubicin, ranimustine, and prednisone (AMP)-vindesine, etoposide, carboplatin, and prednisone (VECP) therapy (VCAP-AMP-VECP) or CHOP-VMMV therapy (n=31)], and showed median survival durations of 16 and 277 days, respectively. Subsequent to the initial treatment, HSCT (n=6) was performed for certain patients, thus revealing that two-thirds (n=4) relapsed, and one-third (n=2) survived for 131 days and 203 days, respectively.
The relapsed ATL patients were treated with conventional salvage therapy (n=29) or anti-CC chemokine receptor 4 antibody (mogamulizumab) (n=3). The patients treated with mogamulizumab demonstrated complete response (2) and partical response (1) with short duration periods of 82 days, 83 days, and 192 days, respectively.
Among the five long-term survivors (>5 years) who received chemotherapy, most showed a low and intermediate risk according to the ATL prognostic index.
Conclusion In our study, the overall survival of ATL remains poor due to the advanced age of the patients at diagnosis, a high proportion of patients receiving palliative therapy, and a small proportion of long-term survivors receiving chemotherapy and undergoing HSCT. This study illustrates the current clinical features, treatment strategies, and outcomes in clinical practice.

• 出版日期2015
• 单位RIKEN