Characteristics, outcomes, prognostic factors and treatment of patients with T-cell prolymphocytic leukemia (T-PLL)

P. Jain, E. Aoki, M. Keating, W. G. Wierda, S. O'Brien, G. N. Gonzalez, A. Ferrajoli, N. Jain, P. A. Thompson, E. Jabbour, R. Kanagal-Shamanna, S. Pierce, A. Alousi, C. Hosing, I. Khouri, Z. Estrov, J. Cortes, H. Kantarjian, F. Ravandi, T. M. Kadia

Research output: Contribution to journalArticle

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.

Patients and 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.

Conclusion: Outcomes in T-PLL remain poor. Multicenter collaborative effort is required to conduct prospective studies.

Original languageEnglish (US)
Pages (from-to)1554-1559
Number of pages6
JournalAnnals of oncology : official journal of the European Society for Medical Oncology
Volume28
Issue number7
DOIs
StatePublished - Jul 1 2017
Externally publishedYes

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T-Cell Prolymphocytic Leukemia
Therapeutics
Pentostatin
Confidence Intervals
Hemoglobins
Survival
L-Lactate Dehydrogenase
Disease-Free Survival
Chromosomes, Human, Pair 14
Stem Cell Transplantation
Pleural Effusion
Rare Diseases
Karyotype

Keywords

  • prolymphocytes
  • prolymphocytic leukemia
  • T-PLL

ASJC Scopus subject areas

  • Hematology
  • Oncology

Cite this

Characteristics, outcomes, prognostic factors and treatment of patients with T-cell prolymphocytic leukemia (T-PLL). / Jain, P.; Aoki, E.; Keating, M.; Wierda, W. G.; O'Brien, S.; Gonzalez, G. N.; Ferrajoli, A.; Jain, N.; Thompson, P. A.; Jabbour, E.; Kanagal-Shamanna, R.; Pierce, S.; Alousi, A.; Hosing, C.; Khouri, I.; Estrov, Z.; Cortes, J.; Kantarjian, H.; Ravandi, F.; Kadia, T. M.

In: Annals of oncology : official journal of the European Society for Medical Oncology, Vol. 28, No. 7, 01.07.2017, p. 1554-1559.

Research output: Contribution to journalArticle

Jain, P, Aoki, E, Keating, M, Wierda, WG, O'Brien, S, Gonzalez, GN, Ferrajoli, A, Jain, N, Thompson, PA, Jabbour, E, Kanagal-Shamanna, R, Pierce, S, Alousi, A, Hosing, C, Khouri, I, Estrov, Z, Cortes, J, Kantarjian, H, Ravandi, F & Kadia, TM 2017, 'Characteristics, outcomes, prognostic factors and treatment of patients with T-cell prolymphocytic leukemia (T-PLL)', Annals of oncology : official journal of the European Society for Medical Oncology, vol. 28, no. 7, pp. 1554-1559. https://doi.org/10.1093/annonc/mdx163
Jain, P. ; Aoki, E. ; Keating, M. ; Wierda, W. G. ; O'Brien, S. ; Gonzalez, G. N. ; Ferrajoli, A. ; Jain, N. ; Thompson, P. A. ; Jabbour, E. ; Kanagal-Shamanna, R. ; Pierce, S. ; Alousi, A. ; Hosing, C. ; Khouri, I. ; Estrov, Z. ; Cortes, J. ; Kantarjian, H. ; Ravandi, F. ; Kadia, T. M. / Characteristics, outcomes, prognostic factors and treatment of patients with T-cell prolymphocytic leukemia (T-PLL). In: Annals of oncology : official journal of the European Society for Medical Oncology. 2017 ; Vol. 28, No. 7. pp. 1554-1559.
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TY - JOUR

T1 - Characteristics, outcomes, prognostic factors and treatment of patients with T-cell prolymphocytic leukemia (T-PLL)

AU - Jain, P.

AU - Aoki, E.

AU - Keating, M.

AU - Wierda, W. G.

AU - O'Brien, S.

AU - Gonzalez, G. N.

AU - Ferrajoli, A.

AU - Jain, N.

AU - Thompson, P. A.

AU - Jabbour, E.

AU - Kanagal-Shamanna, R.

AU - Pierce, S.

AU - Alousi, A.

AU - Hosing, C.

AU - Khouri, I.

AU - Estrov, Z.

AU - Cortes, J.

AU - Kantarjian, H.

AU - Ravandi, F.

AU - Kadia, T. M.

PY - 2017/7/1

Y1 - 2017/7/1

N2 - 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.Patients and 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.Conclusion: Outcomes in T-PLL remain poor. Multicenter collaborative effort is required to conduct prospective studies.

AB - 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.Patients and 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.Conclusion: Outcomes in T-PLL remain poor. Multicenter collaborative effort is required to conduct prospective studies.

KW - prolymphocytes

KW - prolymphocytic leukemia

KW - T-PLL

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U2 - 10.1093/annonc/mdx163

DO - 10.1093/annonc/mdx163

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C2 - 28379307

AN - SCOPUS:85041248150

VL - 28

SP - 1554

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JO - Annals of Oncology

JF - Annals of Oncology

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