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

被引：55

作者：

Jain, P. ^{[1
]}

Aoki, E. ^{[1
]}

Keating, M. ^{[1
]}

Wierda, W. G. ^{[1
]}

O'Brien, S. ^{[2
]}

Gonzalez, G. N. ^{[3
]}

Ferrajoli, A. ^{[1
]}

Jain, N. ^{[1
]}

Thompson, P. A. ^{[1
]}

Jabbour, E. ^{[1
]}

Kanagal-Shamanna, R. ^{[4
]}

Pierce, S. ^{[1
]}

Alousi, A. ^{[5
]}

Hosing, C. ^{[5
]}

Khouri, I. ^{[5
]}

Estrov, Z. ^{[1
]}

Cortes, J. ^{[1
]}

Kantarjian, H. ^{[1
]}

Ravandi, F. ^{[1
]}

Kadia, T. M. ^{[1
]}

机构：

[1] MD Anderson Canc Ctr, Dept Leukemia, Houston, TX USA

[2] UC Irvine, Chao Family Comprehens Canc Ctr, Div Hematol Oncol, Irvine, CA USA

[3] MD Anderson Canc Ctr, Dept Biostat, Houston, TX USA

[4] MD Anderson Canc Ctr, Dept Hematopathol, Houston, TX USA

[5] MD Anderson Canc Ctr, Dept Stem Cell Transplantat, Houston, TX USA

来源：

ANNALS OF ONCOLOGY | 2017年 / 28卷 / 07期

关键词：

T-PLL; prolymphocytic leukemia; prolymphocytes; ATM GENE; TRANSPLANTATION; MUTATIONS; SURVIVAL; ALEMTUZUMAB; JAK3; CHEMOTHERAPY; LYMPHOMA;

D O I：

10.1093/annonc/mdx163

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

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 beta 2M >= 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.

引用

页码：1554 / 1559

页数：6

共 36 条

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