Are double-hit lymphoma patients being adequately evaluated for CNS disease?

Abstract

e19036 Background: Double hit lymphoma (DHL) is a high-grade lymphoma with a propensity to involve the central nervous system (CNS) compared to the typically less aggressive diffuse large B cell lymphoma (DLBCL). Guidelines recommend considering cerebrospinal fluid (CSF) evaluation and prophylaxis per institution guidelines in DHL patients (pts). It is not clear when this evaluation should occur and what prophylaxis should be delivered. Methods: We performed an IRB granted retrospective analysis of pts with de novo DHL based on assessing high-grade lymphoma FISH panels treated within the Advocate Health Care System. The pt’s age, IPI score, CNS-IPI score, systemic treatment, CSF evaluation, CNS prophylaxis or treatment, CNS event (yes/no), progression free survival and overall survival were recorded. Results: Thirty-one pts were evaluated. Seventeen were male. Median age was 72 years (range: 40 to 89). Fifteen were stage IV. Median IPI score was 3 and CNS IPI was 3. Fifteen pts had CSF evaluated within their disease course. Fifteen pts received CNS prophylaxis or treatment including intrathecal (IT) methotrexate (n = 10), IT methotrexate plus hydrocortisone (n = 2), IT cytarabine (n = 1), IT methotrexate and systemic methotrexate (n = 1), or only systemic methotrexate (n = 1). The number of patients with a CNS event during their disease course was 3 with all 3 having leptomeningeal involvement. These 3 pts had CNS IPI scores > 4. Two had CSF involvement at diagnosis. One died at 2 months; the other is alive and disease free at 44 months. The third pt developed CSF disease at relapse and ultimately died at 12 months. This pt had previously received IT methotrexate. For all pts, the median disease free survival (DFS) was 8.1 months; median survival was 11 months and 3-year DFS was 45%. Conclusions: Our small, but extensive review of community oncologists treating DHL patients showed there was a lack of CSF evaluation in these high-risk patients with a rate of less than 50%. For the patients evaluated, there was significant heterogeneity in CNS prophylaxis. The rate of 10% of patients having CNS disease is consistent with other reports of high-risk lymphoma and CNS relapse suggesting all DHL patients should have CNS prophylaxis.