Central nervous system involvement in T-cell lymphoma: A single center experience

Abstract

Background We characterized the incidence of central nervous system (CNS) involvement, risk factors and outcome in a large single institution dataset of peripheral T cell lymphoma (PTCL). Methods Retrospective review of the PTCL database at Memorial Sloan Kettering Cancer Center. We identified 231 patients with any subtype of PTCL between 1994–2011 with a minimum 6 months of follow-up or an event defined as relapse or death. Results Histologies included peripheral T cell lymphoma – no not otherwise specified (PTCL-NOS) (31.6%), angioimmunoblastic (16.9%), anaplastic large cell lymphoma (ALCL), ALK- (12.1%), ALCL, ALK+ (6.1%), extranodal NK/T-cell lymphoma (7.4%), adult T-cell leukemia/lymphoma (ATLL) (7.4%), and transformed mycosis fungoides (8.7%). 17 patients had CNS disease (7%). Fifteen had CNS involvement with PTCL and two had diffuse large B cell lymphoma and glioblastoma. Median time to CNS involvement was 3.44 months (0.16 to 103.1). CNS prophylaxis was given to 24 patients (primarily intrathecal methotrexate). Rates of CNS involvement were not different in patients who received prophylaxis. Univariate analysis identified stage III-IV, bone marrow involvement, >1 extranodal site and ATLL as risk factors for CNS disease. On multivariate analysis, >1 extranodal site and international prognostic index (IPI) ≥ 3 were predictive for CNS involvement. The median survival of patients with CNS involvement was 2.63 months (0.10 to 75). Conclusions Despite high relapse rates, PTCL, except ATLL, carries a low risk of CNS involvement. Prognosis with CNS involvement is poor and risk factors include: >1 extra nodal site and IPI ≥ 3.

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

“…CNS relapse in patients with PTCL is considered a very rare event. In this study, we showed that >1 site of extranodal involvement is significantly associated with risk of CNS relapse, which is in keeping with the results from earlier studies [ 5 – 7 ]. Interestingly, we also found that risk may vary by histologic type; patients with ALK+ ALCL had higher risk of CNS relapse compared to other types, particularly AITL.…”

“…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 ]. Most recently, Gurion and colleagues presented data from Memorial Sloan Kettering Cancer Center, showing that 6.5% (15/231) of patients with PTCL had CNS involvement with a median time to CNS disease of 3.4 months, although again the authors included patients who had CNS involvement at the time of diagnosis as well as patients with ATLL; the latter group is well known to have high chance of CNS relapse [ 5 ].…”

“…Central nervous system (CNS) involvement of lymphoma is an especially challenging situation. Several studies have analyzed CNS involvement and relapse in patients with PTCL and reported that risk of CNS relapse ranged from 2–6% and that survival outcome after CNS relapse was only 1–7 months [ 5 – 7 ]. These studies have identified some risk factors for CNS relapse in PTCL, such as elevated serum lactate dehydrogenase (LDH), extranodal involvement >1 site and high International Prognostic Index (IPI) score.…”

68‐Year Old Woman with Multiple Sclerosis, Cutaneous T‐Cell Lymphoma and Seizures.

“…CNS involvement by peripheral T‐cell lymphomas generally portends poor prognosis. A recent review from a single institution documented 15 of 231 patients with CNS involvement by a peripheral T‐cell lymphoma over a 17‐year time period, of which 8 cases were pathologically confirmed. CNS disease typically presented early (median, 3.44 months from diagnosis of lymphoma to CNS involvement), and survival thereafter was a median of 2.63 months.…”

‘Fatal journey of a cutaneous ulcer’

“…Features associated with a high risk of CNS relapse in diffuse large B-cell lymphoma (DLBCL) include elevated LDH (above institutional normal limit), more than one extranodal site of involvement and specific anatomical sites (testes, breast, paranasal sinus, epidural and bone marrow). Considering its rarity and lack of prospective studies, the British Committee for Standards in Haematology extends the recommendations of using CNS-directed prophylaxis (intrathecal methotrexate (12–15 mg) or systemic methotrexate (3–5 g/m²)) in high-risk DLBCL to PTCL as well 2. Isolated CNS relapse in a case of primary cutaneous ALCL is extremely uncommon.…”