Primary cerebral lymphomatoid granulomatosis as an immune reconstitution inflammatory syndrome in AIDS

Abstract

First described by Liebow [1, 2], lymphomatoid granulomatosis (LG) is an infrequent B-cell multisystemic proliferation related to Epstein–Barr Virus (EBV) [3]. The lungs are the principal location, but the central nervous system (CNS) may also be affected [4]. Primary cerebral LG is rare [5]. In human immunodeficiency virus (HIV) infected patients, four cases have been reported, only once related to an immune reconstitution inflammatory syndrome (IRIS) after highly active antiretroviral therapy (HAART) [6–9]. Histological findings are essential for diagnosis [10]. We report here a case of primary CNS LG after starting HAART. A 44-year-old woman with a colorectal adenocarcinoma treated with surgery and chemotherapy was admitted to our hospital because of Pneumocystis Jirovecii pneumonia and diagnosed with HIV infection. CD4? count was five cells/ ll and HIV viral load 5.17log. HAART was initiated with efavirenz 600 mg, emtricitabine 200 mg and tenofovir 300 mg daily. She progressively developed confusion, disorientation, memory loss and mild aphasia. Magnetic resonance imaging (MRI) showed a diffuse, periventricular leukoencephalopathy with multiple hypointense nonenhancing areas in T1-weighted sequences. Cerebrospinal fluid (CSF) contained proteins of 1.12 g/l, 3 cells/mm and normal glucose (51 mg/dl). CSF polymerase chain reaction (PCR) for herpes viruses and polyomaviruses, serology for toxoplasma and syphilis, CSF and urine PCR and culture for Mycobacterium tuberculosis were negative. A total body tomography did not reveal other lesions. One month later she developed a right arm paresis and her aphasia worsened, presenting difficulties in understanding verbal commands and in repeating short sentences, a nomination deficit and frequent paraphasias. MRI showed leukoencephalopathy progression with a left frontal mass lesion (Fig. 1). CSF examination yielded two cells/mm, glucose of 45 mg/dl, and proteins of 2 g/l. CSF PCR for herpes viruses, polyomaviruses and Mycobacterium tuberculosis, rapid plasma reagin, cryptococcal antigen, and cytology