title: Characteristics, outcomes, prognostic factors and treatment of patients with T-cell prolymphocytic leukemia (T-PLL).
abstract: BACKGROUND
  T-cell prolymphocytic leukemia (T-PLL) is a rare and aggressive disease. In this study, we report our experience from 119 patients with T-PLL.
  
  
  METHODS
  We reviewed the clinico-pathologic records of 119 consecutive patients with T-PLL, who presented to our institution between 1990 and 2016.
  
  
  RESULTS
  One hundred and nineteen patients with T-PLL were analysed. Complex karyotype and aberrations in chromosome 14 were seen in 65% and 52% patients, respectively. Seventy-five patients (63%) were previously untreated and 43 (37%) were initially treated outside our institution. Sixty-three previously untreated patients (84%) received frontline therapies. Overall, 95 patients (80%) have died. Median overall survival (OS) from diagnosis was 19 months [95% confidence interval (CI) 16-26 months]. Using recursive partitioning (RP), we found that patients with hemoglobin < 9.3 g/dl, lactate dehydrogenase (LDH) ≥ 1668 IU/l, white blood cell  ≥ 208 K/l and β2M ≥ 8 mg/l had significantly inferior OS and patients with hemoglobin < 9.3 g/dl had inferior progression-free survival (PFS). In multivariate analysis, we identified that presence of pleural effusion [hazard ratio (HR) 2.08 (95% CI 1.11-3.9); P = 0.02], high LDH (≥ 1668 IU/l) [HR 2.5 (95% CI 1.20-4.24); P < 0.001)], and low hemoglobin (< 9.3 g/dl) [HR 0.33 (95% CI 0.14-0.75); P = 0.008] were associated with shorter OS. Fifty-five previously untreated patients received treatment with an alemtuzumab-based regimen (42 monotherapy and 13 combination with pentostatin). Overall response rate, complete remission rate (CR) for single-agent alemtuzumab and alemtuzumab combined with pentostatin were 83%, 66% and 82%, 73% respectively. In patients who achieved initial CR, stem cell transplantation was not associated with longer PFS and OS.
  
  
  CONCLUSIONS
  Outcomes in T-PLL remain poor. Multicenter collaborative effort is required to conduct prospective studies.
authors.affiliation: Department of Leukemia, The MD Anderson Cancer Center, Houston.
authors.forename: P
authors.initials: P
authors.lastname: Jain
authors.affiliation: Department of Leukemia, The MD Anderson Cancer Center, Houston.
authors.forename: E
authors.initials: E
authors.lastname: Aoki
authors.affiliation: Department of Leukemia, The MD Anderson Cancer Center, Houston.
authors.forename: M
authors.initials: M
authors.lastname: Keating
authors.affiliation: Department of Leukemia, The MD Anderson Cancer Center, Houston.
authors.forename: W G
authors.initials: WG
authors.lastname: Wierda
authors.affiliation: Division of Hematology/Oncology, Chao Family Comprehensive Cancer Center, UC Irvine, Irvine.
authors.forename: S
authors.initials: S
authors.lastname: O'Brien
authors.affiliation: Departments of Biostatistics.
authors.forename: G N
authors.initials: GN
authors.lastname: Gonzalez
authors.affiliation: Department of Leukemia, The MD Anderson Cancer Center, Houston.
authors.forename: A
authors.initials: A
authors.lastname: Ferrajoli
authors.affiliation: Department of Leukemia, The MD Anderson Cancer Center, Houston.
authors.forename: N
authors.initials: N
authors.lastname: Jain
authors.affiliation: Department of Leukemia, The MD Anderson Cancer Center, Houston.
authors.forename: P A
authors.initials: PA
authors.lastname: Thompson
authors.affiliation: Department of Leukemia, The MD Anderson Cancer Center, Houston.
authors.forename: E
authors.initials: E
authors.lastname: Jabbour
authors.affiliation: Hematopathology.
authors.forename: R
authors.initials: R
authors.lastname: Kanagal-Shamanna
authors.affiliation: Department of Leukemia, The MD Anderson Cancer Center, Houston.
authors.forename: S
authors.initials: S
authors.lastname: Pierce
authors.affiliation: Stem Cell Transplantation, The MD Anderson Cancer Center, Houston, USA.
authors.forename: A
authors.initials: A
authors.lastname: Alousi
authors.affiliation: Stem Cell Transplantation, The MD Anderson Cancer Center, Houston, USA.
authors.forename: C
authors.initials: C
authors.lastname: Hosing
authors.affiliation: Stem Cell Transplantation, The MD Anderson Cancer Center, Houston, USA.
authors.forename: I
authors.initials: I
authors.lastname: Khouri
authors.affiliation: Department of Leukemia, The MD Anderson Cancer Center, Houston.
authors.forename: Z
authors.initials: Z
authors.lastname: Estrov
authors.affiliation: Department of Leukemia, The MD Anderson Cancer Center, Houston.
authors.forename: J
authors.initials: J
authors.lastname: Cortes
authors.affiliation: Department of Leukemia, The MD Anderson Cancer Center, Houston.
authors.forename: H
authors.initials: H
authors.lastname: Kantarjian
authors.affiliation: Department of Leukemia, The MD Anderson Cancer Center, Houston.
authors.forename: F
authors.initials: F
authors.lastname: Ravandi
authors.affiliation: Department of Leukemia, The MD Anderson Cancer Center, Houston.
authors.forename: T M
authors.initials: TM
authors.lastname: Kadia
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use*; D014408:Biomarkers, Tumor / Q000235:genetics; D002869:Chromosome Aberrations; D018450:Disease Progression; D018572:Disease-Free Survival; D006801:Humans; D053208:Kaplan-Meier Estimate; D059785:Karyotype; D015461:Leukemia, Prolymphocytic, T-Cell / Q000235:genetics / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D008499:Medical Records; D015999:Multivariate Analysis; D016016:Proportional Hazards Models; D012008:Recurrence; D012074:Remission Induction; D012189:Retrospective Studies; D012307:Risk Factors; D033581:Stem Cell Transplantation / Q000009:adverse effects / Q000401:mortality; D013781:Texas; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor
keywords: T-PLL; prolymphocytes; prolymphocytic leukemia