Background

Mature nodal or extranodal T-cell and NK-cell NHL are a rare and heterogeneous group of NHL with aggressive behavior and poor clinical outcome. Their incidence varies according to geographical region and racial characteristics. Mexico is included in those countries known to have a high incidence of extranodal T/NK-cell lymphoma, type nasal (NKTCL).

Objective

To evaluate the outcome and prognosis of patients with mature nodal or extranodal T-cell or NK-cell NHL in a single institution in Mexico City.

Methods

Clinicopathological characteristic, treatment, outcome, and prognosis of patients admitted to our institution between August, 1991 and December 2009 were analyzed. Prognostic Index T-cell (PIT) was used in all subtypes of lymphomas except in NKTCL subtype. All tissue biopsies and immunophenotypic markers were reviewed by an expert hematopathologist and reclassified according to the WHO 2008 classification. Univariate analysis using log-rank test was used to determine the correlation between clinical features and overall survival (OS). Multivariate analysis using Cox proportional hazard models were performed. A p value < 0.05 was considered significant.

Results

A total of 67 patients were analyzed. Median age was 37 years. B symptoms were presented in 83.6%, 74.6% had at least one site with extranodal disease, 73.1% advanced clinic stage, 32.8% high risk by International Prognostic Index (IPI) and 47.5% high risk by PIT. According to WHO 2008 classification the most common subtype was peripheral T-cell lymphoma not otherwise (PTCL NOS) specified in 38 patients (56.7 %), angioimmunoblastic T-cell lymphoma (AITL) and NKTCL were the second most common subtypes with 8 cases in each group (11.9 %), anaplastic large cell lymphoma (ALCL) kinase-positive (ALK-positive) was identified in 3 patients (4.5 %), ALCL ALK-negative in 2 cases (3.0 %), lymphoblastic lymphoma and subcutaneous panniculitis-like T-cell lymphoma (SPTCL) with 3 patients in each group (4.5 %), hepatosplenic T-cell lymphoma (HSTL) and aggressive NK-cell leukemia with one case in each group (1.5 %). CHOP-like therapy was used in 71.6 % of patients. Nine percent of patients did not receive treatment. The response was evaluated in 53 patients in whom overall response was 71.7 % with 44.8 % achieving complete remission (CR). Median OS was 2760 days (CI 95 % 1153.145-4366.855). Histopathology subtype did not predict OS. Both prognostic scores, IPI and PIT, were able to identify 4 groups of patients with different outcomes. The analysis failed to demonstrate any advantage of adding etoposide to the chemotherapy schedule. Multivariate analysis showed that, IPI, PIT, and CR were predictive for OS (Table 1).

Table 1

Univariate and multivariate analysis for overall survival

UnivariateMultivariate
VariableValue pHazard ratioCI 95.0%Value pHazard ratioCI 95.0%
Beta 2 microglobulin 0.003 1.346 1.139-1.592    
Bone marrow involvement 0.038 2.321 1.057-5.098    
Ann Arbor staging <0.0001 2.283 1.299-4.013    
ECOG <0.0001 2.059 1.477-2.872    
Extranodal disease1 0.012 2.905 1.355-6.322    
International Prognostic Index <0.0001 3.213 2.072-4.982 0.003 2.289 1.320-3.969 
Prognostic Index T-cell <0.0001 2.541 1.564-4.129 0.014 2.992 1.252-7.151 
Treatment protocols2 0.014 1.738 1.116-2.706    
CR not achieved 0.002 2.244 1.334-3.773 0.022 2.304 1.128-4.704 
UnivariateMultivariate
VariableValue pHazard ratioCI 95.0%Value pHazard ratioCI 95.0%
Beta 2 microglobulin 0.003 1.346 1.139-1.592    
Bone marrow involvement 0.038 2.321 1.057-5.098    
Ann Arbor staging <0.0001 2.283 1.299-4.013    
ECOG <0.0001 2.059 1.477-2.872    
Extranodal disease1 0.012 2.905 1.355-6.322    
International Prognostic Index <0.0001 3.213 2.072-4.982 0.003 2.289 1.320-3.969 
Prognostic Index T-cell <0.0001 2.541 1.564-4.129 0.014 2.992 1.252-7.151 
Treatment protocols2 0.014 1.738 1.116-2.706    
CR not achieved 0.002 2.244 1.334-3.773 0.022 2.304 1.128-4.704 
1

HR is greater with more number of extranodal sites involved 3 > 2 > 1.

2

HR is greater when not received treatment. CR: complete response.

Conclusion

Previous publications in Mexican population, with larger number of patients included, were particularly focus on clinical characteristics and prognosis of NKTCL. Our series provides data of mature nodal or extranodal T-cell and NK-cell NHL in Mexico. The current study confirms the poor prognosis of aggressive forms of mature nodal or extranodal T-cell and NK-cell NHL regardless of the chemotherapy schedule employed.

Disclosures:

No relevant conflicts of interest to declare.

Author notes

*

Asterisk with author names denotes non-ASH members.

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