Lymphomatoid Granulomatosis of Central Nervous System and Lung Driven by Epstein Barr Virus Proliferation: Successful Treatment with Rituximab

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Ruben Fernandez *
Esther Gonzalez
Almudena Fernandez
Ana Pilar Gonzalez
Juan Manuel Sancho
Francisco Dominguez
Carmen Fernandez
(*) Corresponding Author:
Ruben Fernandez | rubenfalva@gmail.com

Abstract

Lymphomatoid granulomatosis (LYG) is a very rare Epstein-Barr virus (EBV) associated B-cell lymphoproliferative disorder. We report the case of a 41-year-old man who presented with fever and respiratory symptoms. Computed tomography showed multiple nodules in both lung fields. Polymerase chain reaction (PCR) analysis of the bronchoalveolar lavage was positive for EBV and biopsy of lung node yielded a diagnosis of LYG, grade III. Shortly after initiation of treatment with chemotherapy, neurologic deterioration appeared. Radiologic findings revealed hydrocephalus and PCR analysis of the cerebrospinal fluid (CSF) was positive for EBV. Treatment with intravenous rituximab led to rapid reduction of EBV load in CSF, along with radiological and clinical improvement. After completion of treatment with immunochemotherapy, an autologous stem cell transplantation was performed. Patient stays in remission one year after diagnosis.

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Author Biographies

Ruben Fernandez, Hospital de Cabueñes

Department of Hematology, MD, PhD

Esther Gonzalez

Department of Hematology, MD, PhD

Almudena Fernandez

Department of Hematology, MD, PhD.

Ana Pilar Gonzalez

Stem Cell Transplant Unit, MD, PhD.

Juan Manuel Sancho

Clinical Hematology Department, MD, PhD

Francisco Dominguez

Department of Pathology, MD, PhD

Carmen Fernandez

Department of Hematology, MD, PhD