Leptomeningeal involvement in B-cell chronic lymphocytic leukemia: a case report and review of the literature

Abstract

Background: Central nervous system involvement is considered a rare complication of chronic lymphocytic leukemia, and so there is the risk of being overlooked.

Case presentation: We report a case of central nervous system involvement in a 75-year-old mulatto woman with chronic lymphocytic leukemia after 5 years of follow-up and a literature review on the subject. The clinical course, treatment and outcome are described. A systematic, meticulous and comprehensive analysis of existing publications regarding chronic lymphocytic leukemia with central nervous system involvement was performed.

Conclusion: We concluded that central nervous system involvement of chronic lymphocytic leukemia is probably not associated with any evident risk factors. Diagnostic approach differs by institutions but often includes imaging, morphology and flow cytometry. Resolution of central nervous system symptoms can usually be accomplished with intrathecal chemotherapy or irradiation followed by systemic treatment. The recognition of this entity by clinicians could lead to early detection and treatment, resulting in better outcomes in this rare complication.

Figure 1

Cerebrospinal fluid and peripheral blood immunophenotyping: flow cytometric dot plots of cerebrospinal fluid (A, C and D) and peripheral blood (B) demonstrating CLL populations. The units on the x- and y-axes are fluorescent intensitity. (A and B) CD5 XCD19 in cerebrospinal fluid and peripheral blood cells, respectively. Four colour cytometry was performed using FACSCalibur (BD Biosciences) flow cytometry. The minority of cerebrospinal fluid cells were positive to double staining showing in circle of the panel A. Panel C shows monoclonality to Ig lambda.

Figure 2

Flow diagram representing the selection studies.