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Frontiers of Medicine

ISSN 2095-0217

ISSN 2095-0225(Online)

CN 11-5983/R

Postal Subscription Code 80-967

2018 Impact Factor: 1.847

Front. Med.    2015, Vol. 9 Issue (4) : 468-477    https://doi.org/10.1007/s11684-015-0419-6
RESEARCH ARTICLE
Clinical characteristics and prognostic factors of patients with mature T-cell lymphoid malignancies: a single-institution study of 225 cases
Wen Xue1,Yan Sheng1,Xiangqin Weng1,Yongmei Zhu1,Yan Zhao1,Pengpeng Xu1,Xiaochun Fei2,Xiaoyan Chen2,Li Wang1,*(),Weili Zhao1,*()
1. State Key Laboratory of Medical Genomics, Shanghai Institute of Hematology, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China
2. Department of Pathology, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China
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Abstract

Mature T-cell lymphoid malignancies comprise a group of heterogeneous diseases that vary in clinicopathological features, biological behavior, treatment response, and prognosis. Bone marrow (BM) infiltration is more commonly present in mature T-cell lymphoid malignancies compared with their B-cell counterparts and hence important for differential diagnosis. In this study, clinical characteristics and prognostic factors were analyzed in 225 patients with mature T-cell lymphoid malignancies treated in a single institution. These included 29 cases of T-cell lymphoproliferative disorders (T-LPD, all with BM infiltration) and 196 cases of T-/natural-killer-cell lymphoma (T/NKCL, 56 with BM infiltration and 140 without BM infiltration). The estimated 5-year overall survival (OS) rates of T-LPD and T/NKCL were 96.6% and 37.3%, respectively. T-LPD patients were less likely to exhibit poor performance status, advanced disease stage, presence of B symptoms, or abnormal level of serum β-2 microglobulin. With similar pathological characteristics, T/NKCL patients with BM infiltration showed significantly lower response rates and shorter OS than those without BM infiltration (P = 0.0264 and P<0.0001, respectively). Multivariate analysis indicated that poor performance status, advanced disease stage, elevated serum lactate dehydrogenase level, and BM involvement were independent unfavorable prognostic factors. The Glasgow Prognostic Score may be more efficient than the International Prognostic Index in predicting disease outcome in T/NKCL. In conclusion, clinical characteristics may be useful in more effectively stratifying patients with mature T-cell lymphoid malignancies.

Keywords mature T-cell lymphoid malignancies      clonal T-cell population      bone marrow infiltration      prognostic factors     
Corresponding Author(s): Li Wang,Weili Zhao   
Just Accepted Date: 29 September 2015   Online First Date: 11 November 2015    Issue Date: 26 November 2015
 Cite this article:   
Wen Xue,Yan Sheng,Xiangqin Weng, et al. Clinical characteristics and prognostic factors of patients with mature T-cell lymphoid malignancies: a single-institution study of 225 cases[J]. Front. Med., 2015, 9(4): 468-477.
 URL:  
https://academic.hep.com.cn/fmd/EN/10.1007/s11684-015-0419-6
https://academic.hep.com.cn/fmd/EN/Y2015/V9/I4/468
Characteristics T/NKCL n = 196 (%) T-LPD n = 29 (%) P value
Age (year)
≥60 80 (40.8) 15 (51.7) 0.3155
<60 116 (59.2) 14 (48.3)
Gender
Male 131 (66.8) 19 (65.5) 1.0000
Female 65 (33.2) 10 (35.5)
Performance status (ECOG)
0−1 83 (42.3) 28 (96.6) <0.0001
2−4 113 (57.7) 1 (3.4)
B symptoms
Yes 143 (73.0) 3 (10.3) <0.0001
No 53 (27.0) 26 (89.7)
No. of extranodal involvement
0−1 148 (75.5) 21 (72.4) 0.8181
>1 48 (24.5) 8 (27.6)
Serum lactic dehydrogenase
Normal 90 (45.9) 17 (58.6) 0.2345
Elevated 106 (54.1) 12 (41.4)
Serum β-2 microglobulin
Normal 124 (63.3) 25 (86.2) 0.0192
Elevated 72 (36.7) 4 (13.8)
Serum C-reactive protein
Normal 103 (52.6) 16 (55.2) 0.8439
Elevated 93 (47.4) 13 (44.8)
Albumin (g/L)
≥35 150 (76.5) 25 (86.2) 0.3392
<35 46 (23.5) 4 (13.8)
White blood cell count (109/L)
4−10 112 (57.1) 14 (48.3) 0.4250
Abnormal 84 (42.9) 15 (51.7)
Hemoglobin level (g/dl)
≥100 153 (78.1) 25 (86.2) 0.4626
<100 43 (21.9) 4 (13.8)
Platelet count (1012/L)
≥100 157 (80.1) 25 (86.2) 0.6135
<100 39 (19.9) 4 (13.8)
Serum HBsAg
Positive 27 (13.8) 4 (13.8) 1.0000
Negative 169 (86.2) 25 (86.2)
Serum Epstein-Barr virus antibody
Positive 54 (27.6) 10 (33.5) 0.5087
Negative 142 (74.4) 19 (65.5)
Tab.1  Clinical characteristics of 225 patients with mature T-cell lymphoid malignancies
Characteristics BM involvement P value Characteristics BM involvement P value
Yes n = 56 (%) No n = 140 (%) Yes n = 56 (%) No n = 140 (%)
Age (year) White blood cell count (109/L)
≥60 25 (44.6) 55 (39.3) 0.5224 4−10 24 (42.9) 88 (62.9) 0.0161
<60 31 (55.4) 85 (60.7) Abnormal 32 (57.1) 52 (37.1)
Gender Hemoglobin level (g/dl)
Male 42 (75.0) 89 (63.6) 0.1345 ≥100 37 (66.1) 116 (82.9) 0.0133
Female 14 (25.0) 51 (36.4) <100 19 (33.9) 24 (17.1)
Performance status (ECOG) Platelet count (1012/L)
0−1 13 (23.2) 130 (92.9) <0.0001 ≥100 39 (69.4) 118 (84.3) 0.0286
2−4 43 (76.8) 10 (7.1) <100 17 (30.4) 22 (15.7)
Ann Arbor stage Serum HBsAg
I−II 0 (0.0) 49 (35.0) <0.0001 Positive 9 (16.1) 18 (12.9) 0.6468
III−IV 56 (100.0) 91 (65.0) Negative 47 (83.9) 122 (87.1)
B symptoms Serum Epstein-Barr virus antibody
Yes 29 (51.8) 79 (56.4) 0.6340 Positive 14 (25.0) 40 (28.6) 0.7241
No 27 (48.2) 61 (43.6) Negative 42 (75.0) 100 (71.4)
No. of extranodal involvement International prognostic index
0−1 36 (64.3) 110 (75.6) 0.0465 Low 11 (19.6) 51 (36.4) 0.0276
>1 20 (35.7) 30 (21.4) Low/intermediate 16 (28.6) 47 (33.6)
Serum lactic dehydrogenase Intermediate/high 19 (33.9) 27 (19.3)
Normal 23 (41.1) 67 (47.9) 0.4299 High 10 (17.9) 15 (10.7)
Elevated 33 (58.9) 73 (52.1) Glasgow Prognosis Score
Serum β-2 microglobulin 0 31 (55.4) 50 (35.7) 0.0396
Normal 20 (35.7) 104 (74.3) <0.0001 1 21 (37.5) 73 (52.1)
Elevated 36 (64.3) 36 (25.7) 2 4 (7.1) 17 (12.1)
Serum C-reactive protein Median OS (month) 24 55 0.0016
Normal 18 (32.1) 85 (60.7) 0.0004 CR 20 (35.7) 76 (54.3) 0.0264
Elevated 38 (67.9) 55 (39.3) ORR 29 (51.8) 95 (67.9) 0.0458
Albumin (g/L)
≥35 40 (71.4) 110 (75.6) 0.3509
<35 16 (28.6) 30 (21.4)
Tab.2  Clinical characteristics of 196 T/NKCL patients according to T-cell clonal proliferation in BM
Characteristics T/NKCL with BM infiltration n = 56 (%) T-LPD n = 29 (%) P value
Age (year)
≥60 25 (44.6) 15 (51.7) 0.6478
<60 31 (55.4) 14 (48.3)
Gender
Male 42 (75.0) 19 (65.5) 0.4472
Female 14 (25.0) 10 (35.5)
Performance status (ECOG)
0−1 13 (23.2) 28 (96.6) <0.0001
2−4 43 (76.8) 1 (3.4)
B symptom
Yes 29 (51.8) 3 (10.3) 0.0001
No 27 (48.2) 26 (89.7)
No. of extranodal involvement
0−1 38 (67.9) 21 (72.4) 0.8051
>1 18 (32.1) 8 (27.6)
Serum lactic dehydrogenase
Normal 23 (41.1) 17 (58.6) 0.1695
Elevated 33 (58.9) 12 (41.4)
Serum β-2 microglobulin
Normal 20 (35.7) 25 (86.2) <0.0001
Elevated 36 (64.3) 4 (13.8)
Serum C-reactive protein
Normal 18 (32.1) 16 (55.2) 0.0610
Elevated 38 (67.9) 13 (44.8)
Albumin (g/L)
≥35 40 (71.4) 25 (86.2) 0.1791
<35 16 (28.6) 4 (13.8)
White blood cell count (109/L)
4−10 24 (42.9) 14 (48.3) 0.6527
Abnormal 32 (57.1) 15 (51.7)
Hemoglobin level (g/dl)
≥100 37 (66.1) 25 (86.2) 0.0705
<100 19 (33.9) 4 (13.8)
Platelet count (1012/L)
≥100 38 (67.9) 25 (86.2) 0.0747
<100 18 (32.1) 4 (13.8)
Serum HBsAg
Positive 9 (16.1) 4 (13.8) 1.0000
Negative 47 (83.9) 25 (86.2)
Serum Epstein-Barr virus antibodies
Positive 14 (25.0) 10 (33.5) 0.4472
Negative 42 (75.0) 19 (65.5)
Tab.3  Clinical characteristics of 85 patients with clonal T-cell proliferation in BM
Immuno-phenotype T/NKCL (n = 56) T-LPD (n = 29) P value
PTCL-NOS n = 22 (%) Nasal NKTCL n = 15 (%) AITL n = 8 (%) ALCL n = 6 (%) HSTCL n = 5 (%) Total MF n = 22 (%) T-LGLn = 4 (%) Other T-LPD n = 3 (%) Total
CD2+ 22/22 (100.0) 15/15 (100.0) 7/8 (87.5) 5/6 (83.3) 2/5 (40.0) 51/56 (91.1) 20/22 (90.9) 2/4 (50.0) 0/3 (0.0) 22/29 (75.9) 0.0967
CD3+ 13/22 (59.1) 0/15 (0.0) 3/8 (37.5) 2/6 (33.3) 5/5 (100.0) 23/56 (41.1) 17/22 (77.3) 3/4 (75.0) 3/3 (100.0) 23/29 (79.3) 0.0011
CD4+ 16/22 (72.7) 3/15 (20.0) 6/8 (75.0) 5/6 (83.3) 0/5 (0.0) 30/56 (53.6) 18/22 (81.8) 0/4 (0.0) 3/3 (100.0) 21/29 (72.4) 0.1074
CD5+ 7/22 (31.8) 4/15 (26.6) 8/8 (100.0) 1/6 (16.7) 0/5 (0.0) 20/56 (35.7) 19/22 (86.3) 1/4 (25.0) 3/3 (100.0) 23/29 (79.3) 0.0002
CD7+ 6/22 (27.3) 6/15 (40.0) 4/8 (50.0) 2/6 (33.3) 4/5 (80.0) 22/56 (39.3) 2/22 (9.1) 1/4 (25.0) 0/3 (0.0) 3/29 (10.3) 0.0058
CD8+ 5/22 (22.7) 2/15 (13.3) 1/8 (12.5) 1/6 (16.7) 2/5 (40.0) 11/56 (19.6) 1/22 (4.5) 3/4 (75.0) 1/3 (33.3) 5/29 (17.2) 1
CD56+ 2/22 (9.1) 13/15 (86.7) 0/8 (0.0) 1/6 (16.7) 3/5 (60.0) 19/56 (33.9) 0/22 (0.0) 1/4 (25.0) 0/3 (0.0) 1/29 (3.4) 0.0022
TIA-1+ NA 15/15 (100.0) NA NA NA / NA 4/4 (100.0) NA / /
TCR-β+ 20/22 (90.9) 0/15 (0.0) 8/8 (100.0) 3/6 (50.0) 4/5 (80.0) 35/56 (62.5) 10/22 (45.5) 4/4 (100.0) 2/3 (66.7) 16/29 (55.2) 0.6411
TCR-γ+ 13/22 (59.1) 0/15 (0.0) 3/8 (37.5) 3/6 (50.0) 5/5 (100.0) 24/56 (42.9) 15/22 (68.2) 2/4 (50.0) 1/3 (33.3) 18/29 (62.1) 0.1125
Tab.4  Immunophenotype and TCR rearrangement profile of 85 patients with clonal-T cell proliferation in BM
Fig.1  Overall survival (OS) curves of patients with mature T cell lymphoid malignancies according to histological subtypes with (A) and without bone marrow (BM) infiltration (B).
Fig.2  Overall survival (OS) curves (A) and progression free survival (PFS) curves (B) of patients with T-LPD and T/NKCL, with and without bone marrow (BM) infiltration.
Fig.3  Overall survival (OS) curves according to IPI and GPS risk groups in all T/NKCL patients (A and B), T/NKCL with (C and D) and without bone marrow (BM) infiltration (E and F).
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