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Primary CNS lymphoma in scleroderma: a case series
Danielle M Robinett1, Laura K Hummers1, Meaghan Morris2
1Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Journal of Scleroderma and Related Disorders
|August 5, 2021
Summary
Systemic sclerosis patients on immunosuppression may develop Epstein-Barr Virus positive (EBV+) lymphoma. Decreasing immunosuppression can trigger neurological symptoms, revealing this cancer in systemic sclerosis (SSc) patients.
Area of Science:
- Rheumatology
- Oncology
- Neurology
- Immunology
Background:
- Patients with rheumatologic diseases, such as systemic sclerosis (SSc), face an elevated cancer risk.
- The impact of immunosuppressive therapy on cancer development or detection in SSc is not well understood.
Purpose of the Study:
- To report two cases of primary central nervous system (CNS) lymphoma in patients with SSc.
- To explore the potential role of immunosuppressive therapy withdrawal in the manifestation of neurologic symptoms and subsequent cancer diagnosis.
Main Methods:
- Case series presentation of two patients with SSc.
- Review of clinical course, including immunosuppressive therapy management and development of neurologic symptoms.
- Diagnosis of Epstein-Barr Virus positive (EBV+) diffuse large B cell lymphoma of the CNS.
Main Results:
- Both patients with SSc showed improvement in rheumatic disease with immunosuppression.
- Neurologic symptoms emerged as immunosuppressive therapy was decreased or discontinued.
- Both patients were diagnosed with EBV+ diffuse large B cell lymphoma of the CNS, a previously undescribed occurrence in SSc.
Conclusions:
- Primary CNS lymphoma is a potential, though rare, complication in SSc patients.
- Withdrawal of immunosuppression may trigger an immune response against an underlying tumor or viral antigen, leading to neurologic symptoms and cancer detection.
- Further research is needed to elucidate the relationship between immunosuppression, viral reactivation, and lymphomagenesis in SSc.
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