mycophenolic-acid and Central-Nervous-System-Neoplasms

Primary central nervous system lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin lymphoma and four cases of PCNSL have previously been described in association with mycophenolate mofetil. We report the fifth case of PCNSL in a patient with lupus nephropathy while on mycophenolate mofetil treatment.

Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Biopsy; Central Nervous System Neoplasms; Diffusion Magnetic Resonance Imaging; Fatal Outcome; Female; Humans; Immunocompromised Host; Immunohistochemistry; Immunosuppressive Agents; Lupus Erythematosus, Systemic; Lymphoma, Large B-Cell, Diffuse; Middle Aged; Mycophenolic Acid; Treatment Outcome; Young Adult

Progressive outer retinal necrosis syndrome (PORN) is a severe clinical variant of necrotizing herpetic chorioretinitis, which occurs almost exclusively in patients with advanced acquired immunodeficiency syndrome (AIDS). To date, only a few cases of PORN have been reported in patients, mostly among those who were immunocompromised. To our knowledge, only one case of PORN in a patient with systemic lupus erythematosus (SLE) has been described. We report the case of a 44-year old HIV-negative patient with lupus nephritis, whom was being treated by mycophenolate mophetil (MMF), arechin and prednisone. After 14 months of MMF therapy, the patient revealed PORN symptoms; and several months later, the patient developed Type B primary central nervous system lymphoma (PCNSL). PORN is usually compared to acute retinal necrosis (ARN) syndrome, because of having the same causative agent: varicella zoster virus (VZV). There are also some similarities in clinical findings. Our observation supports the hypothesis that PORN symptoms in HIV-negative patients can be an intermediate form between ARN and PORN, and can vary according to the patient's immune status.

Topics: Adult; Antiviral Agents; Central Nervous System Neoplasms; Chloroquine; Chorioretinitis; Fatal Outcome; Female; Herpes Zoster; Herpesvirus 3, Human; HIV Seronegativity; Humans; Lupus Erythematosus, Systemic; Lymphoma; Magnetic Resonance Imaging; Mycophenolic Acid; Prednisone; Retinal Necrosis Syndrome, Acute; Visual Acuity

Topics: Antibodies, Monoclonal, Murine-Derived; Antineoplastic Combined Chemotherapy Protocols; Bacteremia; Central Nervous System Neoplasms; Drug Administration Schedule; Female; Fever; Fungemia; Humans; Immunocompromised Host; Immunosuppressive Agents; Lupus Nephritis; Lymphoma; Methotrexate; Mycophenolic Acid; Neurotoxicity Syndromes; Neutropenia; Rituximab; Treatment Outcome; Young Adult

Patients with systemic lupus erythematosus (SLE) are susceptible to the development of lymphoproliferative disorders and postulated causes include intrinsic defects in immune surveillance and iatrogenic administration of immunosuppressants. Since the introduction of mycophenolate mofetil (MMF) to the immunosuppressive regimen for the management of post-organ transplantation, there have been reports of primary lymphoma of the central nervous system (PCNSL). MMF has been widely used to treat active SLE patients with Class IV lupus nephritis. In addition to two previously reported cases of PCNSL among SLE patients on long-term MMF, we report a third patient who has been on treatment with MMF for 8 years. The histology showed features compatible with diffuse large B-cell lymphoma with strong immunohistochemical staining for CD20 and positive signal for Epstein-Barr virus (EBV)-encoded RNA by in-situ hybridization that is similar to other case reports, suggesting EBV driven B-cell lymphoproliferative disease. The patient responded to withdrawal of MMF, intravenous methotrexate, rituximab and whole brain radiotherapy. With the increasing use of MMF in active renal as well as non-renal exacerbations of SLE, PCNSL should be included in the differential diagnosis in patients who present with gradual onset of focal neurological deficit.

Topics: Adult; Antibodies, Monoclonal; Antibodies, Monoclonal, Murine-Derived; Antimetabolites, Antineoplastic; Antineoplastic Agents; Central Nervous System Neoplasms; Epstein-Barr Virus Infections; Female; Humans; Immunosuppressive Agents; Lupus Erythematosus, Systemic; Lymphoma, Large B-Cell, Diffuse; Methotrexate; Mycophenolic Acid; Rituximab

Lymphoproliferative disorders are known to complicate immunosuppressive therapy and two cases of primary lymphoma of CNS (PCNSL) have previously been described in association with mycophenolate mofetil (MMF) treatment. We report the third case of PCNSL in a patient with lupus nephropathy while on MMF treatment. PCNSL may be seen more frequently considering the increased use of MMF in immunosuppressant responsive conditions.

Topics: Adult; Central Nervous System Neoplasms; Diffusion Magnetic Resonance Imaging; Fatal Outcome; Female; Humans; Immunosuppression Therapy; Immunosuppressive Agents; Lupus Erythematosus, Systemic; Lymphoma; Mycophenolic Acid