Leukemic Leptomeningeal Involvement in Stage 0 and Stage 1 Chronic Lymphocytic Leukemia

Abstract

Central nervous system (CNS) involvement in early (Rai Stage 0 and Stage 1) chronic lymphocytic leukemia (CLL) is rare, with only five cases reported. We present the sixth reported case, a 77-year-old male with a 4 year history of Stage 0 CLL who presented with sudden onset of diplopia and headache. Workup revealed a leukemic involvement of his CNS and he responded well to treatment with intrathecal (IT) methotrexate. After his third IT treatment, he developed a change in his mental status, consistent with a chemotherapy induced encephalopathy, which was effectively treated with IT hydrocortisone. In addition to the case presentation, we review the previously reported cases in an effort to determine any characteristics common among the Stage 0/1 CLL patients with reported CNS involvement.

Chronic lymphocytic leukemia with central nervous system involvement: report of two cases with a comprehensive literature review

“…Studies attempting to identify risks factors in patients who develop CNS infiltration have failed to identify common characteristics [10,15]. Our literature review corroborates this, as we fail to identify any clear correlation with age, sex, presenting neurologic symptoms, or Rai stage at time of onset.…”

Management of central nervous system involvement in chronic lymphocytic leukaemia: a retrospective cohort of 30 patients

“…A wide range of therapeutic modalities have been reported in sporadic cases (Stagg & Gumbart, 1987;Garicochea et al, 1997;Hagberg et al, 1997;Miller et al, 1997;Elliott et al, 1999;Poplawska-Szczyglowska et al, 1999;Rye et al, 2001;Brick et al, 2002;Remkov a et al, 2003;Knop et al, 2005;Mowatt et al, 2005;Watanabe et al, 2005;Hanse et al, 2008;Kakimoto et al, 2010;Cohen et al, 2012;Imitola et al, 2012;Moazzam et al, 2012;Strati et al, 2016), consisting of either IT, systemic immuno-chemotherapy or radiotherapy, combined or alone. Based on our results, FCR or BR with IT in first line seems an effective therapy, both for non-progressive and progressive CLL.…”

Chronic Lymphocytic Leukemia With Central Nervous System Involvement: A High-Risk Disease?

“…Patients with CLL with early-stage, long-standing, or advanced disease may experience localized leptomeningeal disease. 8,9 However a leptomeningeal dissemination of CLL cells in patients with early-stage disease is extremely rare. Among 67 reported patients with CLL who had CNS involvement, only 1 of 5 patients had CNS disease as a presenting symptom.…”