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)

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

doi:10.2169/internalmedicine.54.1953

ORIGINAL ARTICLES

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)

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

Author information

Noriaki Kawano
Department of Internal Medicine, Miyazaki Prefectural Miyazaki Hospital, Japan
Shuro Yoshida
Department of Internal Medicine, Miyazaki Prefectural Miyazaki Hospital, Japan
Takuro Kuriyama
Department of Internal Medicine, Miyazaki Prefectural Miyazaki Hospital, Japan
Yoshihiro Tahara
Department of Internal Medicine, Miyazaki Prefectural Miyazaki Hospital, Japan
Kiyoshi Yamashita
Department of Internal Medicine, Miyazaki Prefectural Miyazaki Hospital, Japan
Yuri Nagahiro
Department of Psychiatry, Kuranaga Hospital, Japan
Jiro Kawano
Department of Psychiatry, Miyazaki Prefectural Miyazaki Hospital, Japan
Hideki Koketsu
Department of Dermatology, Miyazaki Prefectural Miyazaki Hospital, Japan
Atsushi Toyofuku
Department of Surgery, Miyazaki Prefectural Miyazaki Hospital, Japan
Tatsuya Manabe
Department of Surgery, Miyazaki Prefectural Miyazaki Hospital, Japan
Kiichiro Beppu
Department of Surgery, Miyazaki Prefectural Miyazaki Hospital, Japan
Nobuyuki Ono
Department of Internal Medicine, Miyazaki Prefectural Miyazaki Hospital, Japan
Daisuke Himeji
Department of Internal Medicine, Miyazaki Prefectural Miyazaki Hospital, Japan
Naoko Yokota-Ikeda
Department of Internal Medicine, Miyazaki Prefectural Miyazaki Hospital, Japan
Sanshiro Inoue
Department of Orthopedics, Miyazaki Prefectural Miyazaki Hospital, Japan
Hidenobu Ochiai
Trauma and Critical Care Center, Faculty of Medicine, University of Miyazaki Hospital, Japan
Koh-Hei Sonoda
Department of Ophthalmology, Yamaguchi University Graduate School of Medicine, Japan
Kazuya Shimoda
Devision of Gastroenterology and Hematology, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Japan
Fumihiko Ishikawa
Research Unit for Human Disease Models, RIKEN Research Center for Allergy and Immunology, Japan
Akira Ueda
Department of Internal Medicine, Miyazaki Prefectural Miyazaki Hospital, Japan

Keywords: 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

JOURNAL OPEN ACCESS

2015 Volume 54 Issue 12 Pages 1489-1498

DOI https://doi.org/10.2169/internalmedicine.54.1953

Details

Abstract

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.

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