Journal List > Korean J Hematol > v.40(1) > 1032655

Baek, Park, Choi, Kim, Kim, Kim, Yoon, Park, Kim, Kim, and Heo: Angioimmunoblastic T-cell Lymphoma: Clinical Characteristics and Treatment Outcomes

Abstract

Background

An angioimmunoblastic T-cell lymphoma (AITL) is a rare subtype of lymphoma, accounting for only 1 to 2% of studies on non-Hodgkin's lymphomas. Because of the rarity of this disease, most studies have been small, including cases of various T-cell Non-Hodgkin's Lymphoma (T-NHL). Those patients diagnosed as AITL, during the last 8 years at a single institution (Seoul National University Hospital), were retrospectively analyzed to determine the clinical features and treatment outcomes of AITL.

Methods

All 24 of the patients diagnosed with AITL between February 1995 and February 2003 were included in this retrospective review.

Results

The predominant characteristics of the population were: median age 62 years (range, 32~81); M/F=18/6; nodal involvement 24/24 (100%); extranodal involvement, particularly bone marrow 16/20 (80%); skin involvement 6/24 (25%); B-symptoms 18/24 (75%) and advanced disease (stages III and IV) in 20/24 (83%). Twenty-three of the 24 patients received combination chemotherapy, with 8/23 (35%) of patients obtaining a CR. The median CR duration was 18.1 months. With a median follow-up of 40.9 months, the 5-year OS rate was 28%, with median survival of 8.7 months. According to a univariate analysis, an elevated LDH showed a tendency to negatively influence the survival.

Methods

All 24 of the patients diagnosed with AITL between February 1995 and February 2003 were included in this retrospective review.

Conclusion

The prognosis of AITL is poor compared to other NHL, with a low CR rate and short CR duration and OS. From our data, the CR rate after first- or second-line chemotherapy were low (35%), compared with those previously described in Western reports.

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Fig. 1.
Disease-free survival of the 8 patients who achieved complete remission.
kjh-40-8f1.tif
Fig. 2.
Overall survival of the 24 patinets with AITL.
kjh-40-8f2.tif
Table 1.
Relative frequency of histologic subtypes of NHLs according to REAL classification
Study population Korean (Kang et al)21) (N=802) Korean (Ko et al)22 (N=1,466) International (NHLCP)23 (N=1,378)
B-cell (total) 69.3 74.8 82.1
T-cell (total) 30.7 25.2 17.9
 Peripheral T-cell 26.0 20.0 13.5
 Angiocentric 10.5 8.8 1.4
 Angioimmunoblastic 1.6 1.0 1.2
 PTCL, NOS 13.8 9.4 10.9
 Anaplastic large cell 1.9 1.5 2.4

Modified from J Korean Cancer Assoc 1999;31(4):641-52.20

Table 2.
Clinical characteristics of the AITL patients (N=24)
  N %
Age median: 62yr (32~81)    
 ≤60yr 10 42
 >60yr 14 58
Sex    
 Males 18 75
 Females 6 25
Stage    
 I~II 4 17
 III~IV 20 83
Performance status    
 1~2 18 75
 3~4 6 25
B-symptoms    
 Present 18 75
 Absent 6 25
LN enlargement 24 100
Hepatosplenomegaly 16 66
Skin    
 Rash 5 20
 Nodules 1 4
Extranodal involvement    
 Bone marrow 16/20 80
 Pleural effusion 9 38
 Ascites 3 13
 Cerebrospinal fluid 0 0
IPI    
 Low risk 1 4
 Low-intermediate 3 13
 High-intermediate 7 29
 High 13 54

Available for only 20 patients.

Table 3.
Laboratory findings of the AITL patients (N=24)
  N %
Hemoglobin (g/dL)    
 <10 10 42
 ≥10 14 58
Hypereosinophilia (>500/μL) 8 33
Polyclonal hypergammoglobulinemia (>13g/dL) 8/12 67
LDH (IU/L)    
 Normal (<225) 3 12
 ~300 7 29
 301~400 7 29
 401~500 3 13
 >500 4 17

Available for only 12 patients.

Table 4.
Response according to initial therapy
First-line therapy N CR N (%) PR N (%) Median CR duration Relapse
Month N (%)
Chemotherapy 23 8 (35) 4 (17%)    
 Anthracycline-based 16 8 (50) 2 (13%) 18.1 5/8 (62)
 Others 7 0 (0) 2 (29%)    

CHOP (10/16), COPBLAM-V (6/16)

IMEP (4/7), CVP (3/7). Abbreviations: CR, complete remission; PR, partial remission.

Table 5.
Prognostic factor rs influencing overa all survival
Prognostic factors (N) Overall survival (month) P (Log rank test)
Age
 ≤60yrs (10) 5.9 0.7686
 >60yrs (14) 10.4  
B-symptom
 Absent (18) 4.5 0.8948
 Present (6) 8.7  
Stage    
 I~II (4) 45.5 0.1742
 III~IV (20) 5.9  
IPI
 L-LI (4) 48.2 0.1654
 HI-I (20) 5.1  
LDH
 ≤300IU/L (10) 16.0 0.0485
 >300IU/L (14) 4.5  
Hemoglobin    
 ≥10g/dL (14) 8.7 0.8381
 <10g/dL (10) 5.9  
Extranodal involvement (20)
 Without involvement (2) 45.5 0.4080
Involvement (18) 5.9  
Table 6.
Comparision of patient characteristics and treatment outcomes
Study No of cases N Median age (yrs) Stage III/IV (%) B-sx (+) (%) BM (+) (%) Skin (+) (%) IPI Outcomes
L-LI (%) HI-H (%) CR (%) 5yr-OS (%)
Kang et al.21) 13 59 72 63 18 - 45 54 - 43
Pautier P et al.7) 33 62 94 85 61 48 - - 60 36
Edward RA et al.24) 13 60 77 46 31 - 44 56 90 26
This study 24 61.5 83 75 80 24 17 83 34 28

Abbreviations: IPI, international prognostic index; L-LI, low-low intermediate risk; HI-H, high intermediate-high risk; CR, complete remission; 5yr-OS, 5-year overall survival; -, not reported.

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