Central Nervous System Lymphoma in a Patient with Chronic Lymphocytic Leukemia: A Case Report and Literature Review.

Abdulrahman Albakr,Seema Nagpal,Lauren Maeda,Peyman Samghabadi,Abdulrazag Ajlan,Wajda Alhothali

doi:10.7759/cureus.3660

Abstract

Chronic lymphocytic leukemia (CLL) is the most common type of leukemia that affects older adults in the Western world. Symptomatic nervous system invasion in undiagnosed CLL is rare, poorly understood, challenging to treat, and associated with decreased survival. The average survival of CLL patients with central nervous system (CNS) involvement is 3.79 years as compared to six years in CLL patients without CNS involvement. Autopsy studies demonstrated a high incidence of undiagnosed CLL with CNS involvement, suggesting that CNS involvement is either underdiagnosed or subclinical. Although the most common site of CNS involvement is the leptomeninges, our case demonstrates an extremely rare form of CNS diffuse large B-cell parenchymal involvement in a patient with a concurrent diagnosis of systemic CLL.

Highlights

The incidence of central nervous system (CNS) involvement in chronic lymphocytic leukemia (CLL) ranges from 0.8% to 2% in antemortem studies and up to 7% to 71% of cases diagnosed at autopsy [1,2]
Autopsy studies demonstrated a high incidence of undiagnosed Chronic lymphocytic leukemia (CLL) with CNS involvement, suggesting that CNS involvement is either underdiagnosed or subclinical
The Rai staging system is one of several clinical staging systems that assess the clinical outcome for CLL patients and assigns a score based on the presence or absence of lymphocytosis, lymph node enlargement, splenomegaly, hepatomegaly, anemia, and thrombocytopenia [8]

Summary

Introduction

The incidence of central nervous system (CNS) involvement in chronic lymphocytic leukemia (CLL) ranges from 0.8% to 2% in antemortem studies and up to 7% to 71% of cases diagnosed at autopsy [1,2]. We report a case of CNS lymphocytic intra-parenchymal involvement in a patient with previously undiagnosed CLL. A neurological examination revealed mild dysmetria in the left upper extremity Her white cell count was 25,100 × 1012/l, with 61% lymphocytes. A bone marrow core biopsy was performed due to low white blood cell (WBC) count, which revealed the involvement of a low-grade lymphoid process (Figures 1L-1M). Molecular studies performed on the paraffin-embedded tissue from the brain biopsy revealed an immunoglobulin heavy chain (IGH) rearrangement consistent with a clonal process.

Discussion

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Disclosures