Cerebrospinal Fluid Involvement in Mantle Cell Lymphoma

Abstract

Mantle cell lymphoma (MCL) is an aggressive neoplasm that is incurable by standard therapy. Patients often present with high-stage disease (Stages III-IV) and frequently have involvement at multiple extranodal sites. Although the gastrointestinal tract, spleen, lung, pleura, bone marrow, and peripheral blood are among the most commonly involved tissues, MCL may also disseminate to so-called sanctuary sites including the central nervous system. Despite this, current clinical evaluations do not routinely include assessment of the cerebrospinal fluid (CSF) for lymphomatous infiltrates at presentation, and moreover, only few authors have specifically examined CSF involvement in MCL. In this study, we reviewed the medical records of 108 patients with MCL seen at three centers over a 5-year period to determine the rate of CSF sampling and the frequency of CSF involvement by MCL. The clinical and cytologic characteristics associated with CSF involvement were also studied. Central nervous system (CNS) signs and/or symptoms prompted CSF sampling in 25/108 patients (23%). Specific radiographic abnormalities were present in 9/25 patients (36%). CSF involvement by MCL was identified in 10 of the 25 CSF-sampled patients (40%) by morphology (3 patients), flow cytometry alone (1 patients), or both (6 patients). The CSF-positive cases included two blastoid variants. The CSF cytology of the nine morphologically positive cases was pleomorphic, and prominent cytoplasmic granules were observed in two cases. The overall rate of CSF involvement by MCL among the cohort was 9%, which is comparable to that reported in other selected studies examining this issue.

Central nervous system involvement in mantle cell lymphoma

“…The risk for central nervous system (CNS) infiltration was first reported by Bedotto et al [7] in 1986 and Ellison et al [8], and subsequently confirmed by our own group [9] in a series of 22 patients of whom five (22%) eventually had CNS involvement, and by others [10,11], who reported an overall incidence of 4% and 9%, respectively. However, the incidence, predicting factors, and outcome of CNS involvement in patients with MCL have not been thoroughly investigated in large series of patients from single institutions.…”

Central nervous system prophylaxis in mantle cell lymphoma

“…Beside already mentioned age difference, the GMALL protocol does not encounter CNS disease at presentation among risk classification criteria, whereas the standard risk groups also includes the subset of T-cell leukemia. 5,6 One possible explanation for association found in our study 1 is that higher frequency of toxicity in A934 carriers would lead to more frequent drug withdrawal or dose reduction, which might cause higher frequency of relapse. However, this reduction probably would not be sufficient to explain reduction in event-free survival, and other mechanisms contribute as well.…”

“…For instance, in the United Kingdom, one-third of hematologists administer prophylaxis to MCL patients and only in specific circumstances. 2 Among the many reports of MCL in the literature, 4 concentrated their efforts on direct assessment of CNS involvement in MCL, [3][4][5][6] and all are retrospective (Table 1); therefore, the true incidence is unknown. Interestingly, although most patients with CNS involvement develop symptoms at or after first relapse, not only the blastoid variant MCL had this propensity while having a higher risk.…”

“…4 There are several differences in treatment modalities between childhood ALL DFCI protocol and GMALL adult ALL trial regarding drug type and dose, and schedule of administration. 5,6 Beside noted difference in MTX dose, high-dose MTX in GMALL trial is given at several instances during consolidation phase compared with earlier single administration during remission induction in DFCI protocol.…”

Suboccipital Lateral Injection of Intrathecal Chemotherapy in a Patient with Mantle Cell Lymphoma

“…All patients were treated with intrathecal chemotherapy without response; survival time after diagnosis ranged from 18 to 55 days. Another study of Valdez et al [8] reviewed the medical records of 108 patients with MCL for CNS involvement; the overall rate of positive cytologic findings in the cerebrospinal fluid being 9%. The diagnosis of meningeal manifestations can be based on clinical symptoms, lumbar puncture and magnetic resonance imaging (MRI).…”