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Joint Bone Spine
Sous presse. Epreuves corrigées par l'auteur. Disponible en ligne depuis le dimanche 1 décembre 2019
Doi : 10.1016/j.jbspin.2019.10.005
accepted : 9 October 2019
Epstein-Barr virus-associated primary central nervous system lymphoma in a patient with diffuse cutaneous systemic sclerosis on long-term mycophenolate mofetil
 

Soumya Chatterjee a, , Lilyana Angelov b, Manmeet S. Ahluwalia c, Gabrielle A. Yeaney d
a Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Staff, Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, Ohio 44195, United States 
b Neurological Surgery, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Section of Spinal Radiosurgery and Director of BBTC's Primary CNS Lymphoma Program, Brain Tumor and Neuro-Oncology Center, Cleveland Clinic, Cleveland, Ohio 44195, United States 
c Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Brain Metastasis Research Program, and the Associate Director, Clinical Trials, Operations in the BBTC, Neurological Institute, Cleveland Clinic, Section Head of NeuroOncology Outcomes, Staff, Taussig Cancer Institute, Cleveland Clinic, Cleveland, Ohio 44195, United States 
d Pathology, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Section Head, Ocular Pathology, Division of Neuropathology, Department of Anatomic Pathology, Cleveland Clinic, Cleveland, Ohio 44195, United States 

Corresponding author. Department of Rheumatic and Immunologic Diseases, Orthopaedic and Rheumatologic Institute, Cleveland Clinic, 9500 Euclid Avenue, Desk A50, Cleveland, OH 44195, United States.Department of Rheumatic and Immunologic Diseases, Orthopaedic and Rheumatologic Institute, Cleveland Clinic9500 Euclid Avenue, Desk A50ClevelandOH44195United States
Highlights

Because of a favorable toxicity profile, mycophenolate mofetil is frequently used as long-term immunosuppressive therapy for various autoimmune disorders.
Immunocompromised subjects are prone to develop Epstein Barr virus (EBV)-associated primary central nervous system lymphoma (ePCNSL).
We report the first case of ePCNSL in a patient with diffuse cutaneous systemic sclerosis receiving long-term mycophenolate mofetil.
Patients on long-term mycophenolate mofetil should undergo regular surveillance for neurological deficits so that prompt evaluation and management can be initiated.

The full text of this article is available in PDF format.
Abstract
Background

Epstein Barr virus (EBV)-associated primary central nervous system lymphoma (ePCNSL) is increasingly recognized in immunocompromised subjects, including patients receiving systemic immunosuppressive therapy. Here, we report the first case of primary CNS lymphoma associated with EBV in a patient with diffuse cutaneous systemic sclerosis (dcSSc) receiving long-term mycophenolate mofetil (MMF).

Case report

A 51-year-old female with dcSSc had been on MMF 2 grams daily, which was initiated for a rapidly rising modified Rodnan skin score (mRSS), severe pruritus, and progressive joint contractures. She had an impressive response to this therapy with a significant decrease in her mRSS. Her condition remained stable for the next five years, after which she developed worsening headaches for 2–3 weeks, associated with dizziness, gait instability, and left homonymous hemianopia. MRI scan of the brain revealed a solitary 2.4cm peripherally enhancing right parietal lobe mass. Excised tissue from the right parietal lobe mass showed EBV-associated diffuse large B cell lymphoma. She received four cycles of chemotherapy (high dose methotrexate and rituximab). Currently, her condition is being monitored. Her left homonymous hemianopia persists.

Conclusion

Because of a favorable toxicity profile, MMF is increasingly being used as long-term immunomodulatory therapy for a wide variety of autoimmune disorders. Nevertheless, patients on long-term MMF should still undergo regular CNS surveillance, not only for opportunistic infections but also for opportunistic malignancies such as PCNSL. Progressive focal or non-focal neurological deficits should always raise the alarm. Prompt evaluation and management can prevent irreversible neurological sequelae.

The full text of this article is available in PDF format.

Keywords : Mycophenolate mofetil, Epstein Barr virus, Diffuse large B cell lymphoma, Primary central nervous system lymphoma (PCNSL), Diffuse cutaneous systemic sclerosis (dcSSc)




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