title: 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).
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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Internal Medicine, Miyazaki Prefectural Miyazaki Hospital, Japan.
authors.forename: Noriaki
authors.initials: N
authors.lastname: Kawano
authors.forename: Shuro
authors.initials: S
authors.lastname: Yoshida
authors.forename: Takuro
authors.initials: T
authors.lastname: Kuriyama
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Tahara
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Yamashita
authors.forename: Yuri
authors.initials: Y
authors.lastname: Nagahiro
authors.forename: Jiro
authors.initials: J
authors.lastname: Kawano
authors.forename: Hideki
authors.initials: H
authors.lastname: Koketsu
authors.forename: Atsushi
authors.initials: A
authors.lastname: Toyofuku
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Manabe
authors.forename: Kiichiro
authors.initials: K
authors.lastname: Beppu
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Ono
authors.forename: Daisuke
authors.initials: D
authors.lastname: Himeji
authors.forename: Naoko
authors.initials: N
authors.lastname: Yokota-Ikeda
authors.forename: Sanshiro
authors.initials: S
authors.lastname: Inoue
authors.forename: Hidenobu
authors.initials: H
authors.lastname: Ochiai
authors.forename: Koh-Hei
authors.initials: KH
authors.lastname: Sonoda
authors.forename: Kazuya
authors.initials: K
authors.lastname: Shimoda
authors.forename: Fumihiko
authors.initials: F
authors.lastname: Ishikawa
authors.forename: Akira
authors.initials: A
authors.lastname: Ueda
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000008:administration & dosage; D018450:Disease Progression; D004317:Doxorubicin / Q000008:administration & dosage; D004334:Drug Administration Schedule; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000401:mortality* / Q000473:pathology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000008:administration & dosage; D011379:Prognosis; D012074:Remission Induction; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone