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

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.

Refractory Chronic Lymphocytic Leukemia with Central Nervous System Involvement: A Case Report with Literature Review

“…97. e12701 28 67 F Slurred speech, headache, and left-sided hemiparesis NA NA 14,500 p53 + Surgical resection CSF (DLBCL) Yes HD Mtx IT Mtxand AraC PD Cureus.2018.10. e3660 29 84 F Mild dysmetria in the upper-left extremity NA Rai 0 15,311,000 NA Tumor resection Yes Temozolomide and WBRT PD Ann Indian Acad Neurol. 2018.…”

Extensive Central Nervous System Lymphoma Detected at the Time of Diagnosis of Chronic Lymphocytic Leukemia - An Isolated Central Nervous System Richter’s Syndrome: A Case Report

“…Of the 19 reported isolated CNS RS cases, data were available for 10 patients, including 8 with Binet stage A, 2 with Binet stage B, and no patient with advanced Binet stage. 1,23,25) Our patient had Binet stage A, Rai stage 0 without LN adenopathy, splenomegaly, and thrombocytopenia, but she did not have other suspected clinical RS symptoms, such as fever and elevation of serum LDH level. It may be difficult to suspect the first chance that isolated CNS RS occurred alone without other nodal/extranodal lesion.…”

“…Among the 19 isolated CNS RS cases reported by 2022, four were diagnosed simultaneously with the initial diagnosis of CLL. 1,3,23,25) CNS involvement in CLL/RS can present with visual changes(22%), encephalopathy(29%), and weakness with paresthesia(22%). 18) Symptoms are based on the location of the lesion, both favor to spread via leptomeninges.…”

Chronic Lymphocytic Leukaemia: Rare Involvement of the Central Nervous System

“…Risk factors for CNS involvement in CLL have not been systematically explored [ 2 ] , although the presence of infectious agents can destabilize the blood–brain barrier [ 5 ] . Current knowledge suggests that CNS involvement is not related to disease stage, duration or progression [ 4 ] , or to a high-risk chromosomal abnormality, such as del11p or del111q [ 3 ] .…”

“…Chronic lymphocytic leukaemia (CLL) is the most common adult leukaemia in Western countries and is characterized by the proliferation and accumulation of small mature-appearing lymphocytes in the blood, bone marrow and lymphoid tissues [ 1 ] , the involvement of other locations being rare [ 2 ] . There are few reports on central nervous system (CNS) infiltration by CLL cells [ 3 ] , which can occur at any time during the natural history of the disease and is associated with lower overall survival [ 4 ] . Neurological complications arising from direct leukaemic involvement in the CNS are reported in only 1% of patients with CLL [ 5 – 7 ] .…”