Isolated Intracerebral Diffuse Large B Cell Lymphoma Secondary to Chronic Lymphocytic Leukemia: Case Report and Literature Review

Case Report

Ann Hematol Oncol. 2019; 6(6): 1254.

Isolated Intracerebral Diffuse Large B Cell Lymphoma Secondary to Chronic Lymphocytic Leukemia: Case Report and Literature Review

Liu S1,2, Qian C1,2, Wang M1,2, Zhang L1,2, Xu X³, Sun A1,2 and Huang H1,2*

¹Department of Hematology, The First Affiliated Hospital of Soochow University, China

²Jiangsu Institute of Hematology, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, China

³Department of Radiotherapy, The First Affiliated Hospital of Soochow University, China

*Corresponding author: Haiwen Huang, Department of Hematology, Jiangsu Institute of Hematology, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, The First Affiliated Hospital of Soochow University, NO.188.Shi Zi Street, Suzhou, 215006, China

Received: April 08, 2019; Accepted: May 09, 2019;Published: May 16, 2019

Abstract

Patients with Chronic Lymphocytic Leukemia (CLL) may develop into Diffuse Large B Cell Lymphoma (DLBCL), which has been described as Richter Syndrome (RS). The prognosis of RS is dismal and the median survival ranges from 6 to 8 months. However, Isolated parenchymal DLBCL lesions in the Central Nervous System (CNS) secondary to CLL is extremely rare. Controversy exists as to whether diffuse large B cells of RS were derived from the preexisting CLL or de novo clone. Here we report a case of a 60-year-old male patient who had a 5-month history of CLL without treatment and developed into isolated parenchymal DLBCL in CNS. In this case, we demonstrated that the clone of DLBCL in CNS was originated from unrelated B cells but not from the preexisting CLL cells by Immunoglobulin Heavy chain (IgH) gene analysis.

Keywords: Secondary diffuse large B cell lymphoma; Chronic lymphocytic leukemia; Richter syndrome; Central nervous system; Immunoglobulin heavy chain gene

Introduction

Richter Syndrome (RS) is the development of an aggressive lymphoma in patients with CLL or SLL [1]. In 1928, Dr. Richter firstly reported RS as a secondary large cell immunoblastic lymphoma that developed in a 46-year-old man with Chronic Lymphocytic Leukemia (CLL) [2]. As previously reported, Patients with Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL) have an increased incidence risk of second malignancy and at least 3% of them develop Diffuse Large B Cell Lymphoma (DLBCL) [3]. In fact, DLBCL secondary to CLL is not always derived from the clone of the preexisting B-CLL and Immunoglobulin heavy chain gene (IgH gene) analysis is one of the key method to differentiate the clones [4]. To our knowledge, few investigators discriminated the clones in RS patients. In this case, we reported a patient who has developed DLBCL in brain after the initial diagnosis of CLL and analyzed the clones of DLBCL.

Case Presentation

A 60-year-old man was referred to us because of leukocytosis of 28.6×10^9/L and lymphocytosis of 18.02×10^9/L in the peripheral blood in September 2016. Bone marrow smears were predominantly shown as mature lymphocytoid morphology. The flow cytometric analysis found that 28.6% monoclonal B lymphocytes, and his immunophenotype were as follows: CD5 +, CD19 +, CD20 +, CD23 +, CD22 (dim), FMC-7 (dim) and CD10-. His condition is generally good with no obvious organ enlargement. He was classified as stage CLL according to Rai classification and no therapeutic interventions were undertaken. Five months later, he was admitted to neurosurgery department because of language dysfunction and consciousness disturbance. Magnetic Resonance Imaging (MRI) showed a 2.5×2.8- cm size signal abnormality with peripheral edema in the deep left temporal region, and significant ring enhancement after gadolinium injection (Figure 1). Positron Emission Tomography-Computed Tomography (PET-CT) of whole body was normal except welldefined mass lesions with an increased fluorodeoxyglucose uptake in left temporal area (SUVmax: 10.27) (Figure 2). Tumor excision was performed in March 2017 and histological examination revealed the aggressive diffuse large B-cell lymphoma. The tumor cells expressed CD20, CD79a, PAX-5, CD5, BCL-2, BCL-6, MUM-1, c-myc and p53 but not CD23, CD10, CD2, CD3 or cyclinD1. Particularly, the Ki-67 labeling index is above 80%. Therefore, DLBCL was considered in the central nervous system from these findings.