Central nervous system relapse in peripheral T-cell lymphomas: a Swedish Lymphoma Registry study

Abstract

Key Points• CNS involvement at relapse/ progression in PTCL occurred at a frequency similar to what is seen in aggressive B-cell lymphomas.• Outcome after relapse is generally very poor in patients with PTCL and is not significantly altered by presence of CNS involvement at relapse.Central nervous system (CNS) relapse in non-Hodgkin lymphoma (NHL) carries a very poor prognosis. Risk factors and outcome have been studied in aggressive B-cell lymphomas, but very little is known about the risk in peripheral T-cell lymphoma (PTCL). We aimed at analyzing risk factors for CNS involvement at first relapse or progression, as well as the outcome of these patients, in a large population-based cohort of patients with PTCL. Twenty-eight out of 625 patients (4.5%) developed CNS disease over time. In multivariable analysis, disease characteristics at diagnosis independently associated with an increased risk for later CNS involvement were involvement of more than 1 extranodal site (hazard ratio [HR], 2.60; 95% confidence interval [CI], 1.07-6.29; P 5 .035) and skin (HR, 3.51; 95% CI, 1.26-9.74; P 5 .016) and gastrointestinal involvement (HR, 3.06; 95% CI, 1.30-7.18; P 5 .010). The outcome of relapsed/refractory patients was very poor, and CNS involvement was not associated with a significantly worse outcome compared with relapsed/refractory patients without CNS involvement in multivariable analysis (HR, 1.6; 95% CI, 0.96-2.6; P 5 .074). The results from the present study indicate that CNS relapse in PTCL occurs at a frequency similar to what is seen in aggressive B-cell lymphomas, but the poor outcomes in relapse are largely driven by systemic rather than CNS disease. (Blood. 2015;126(1):36-41)

Adult T-cell leukemia/lymphoma in the Caribbean cohort is a distinct clinical entity with dismal response to conventional chemotherapy

“…The rate of CNS involvement in this population was as high as 33%, supporting the argument for primary CNS prophylaxis at the time of diagnosis. Despite higher rates of CNS dissemination compared to previously reported incidence in other mature T-cell lymphomas (4.5%) [21], mortality is primarily due to uncontrolled systemic disease. Flow cytometry showed a CD4+/CD8-, CD25+ CD7- T-cell population in most patients, reflecting the typical ATLL immunophenotype.…”

The risk of central nervous system relapses in patients with peripheral T-cell lymphoma

“…The 5-year cumulative incidence of CNS relapse was 2.1% in our study. In a study from the Swedish Lymphoma Registry, 4.5% (28/625) of patients developed CNS relapse at a median of 4.3 months after diagnosis [ 6 ]. Yi and colleagues reported an even higher incidence of CNS involvement of 8.8% (20/228) in patients with PTCL at a median time to CNS disease of 6.1 months, though they included patients who had CNS involvement at the time of diagnosis [ 7 ].…”

“…The association between the site of disease involvement and future risk of CNS disease is worth evaluating. The Swedish Lymphoma Registry study identified skin and gastrointestinal tract involvement are significant risk factors for CNS relapse [ 6 ]. Yi and colleagues identified paranasal sinus involvement as a significant risk factor for CNS relapse [ 7 ].…”

Central Nervous System Prophylaxis in Non-Hodgkin Lymphoma: Who, What, and When?

“…Ten patients (1.3 %) had CNS involvement at diagnosis, whilst 31/755 (5.0 %) developed CNS involvement at a median of 4.3 months after diagnosis. The presence of B-symptoms, multiple extranodal sites of involvement, and cutaneous involvement were independent predictors of CNS involvement [89]. Yi et al reported a crude incidence of CNS of 8.8 % in 228 patients with nodal PTCL [90].…”