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Lymphoma in Sjogren's syndrome
N A Pavlidis1, A A Drosos, C Papadimitriou
1Department of Medicine and Pathology, Medical School, University of Ioannina, Greece.
Medical and Pediatric Oncology
|January 1, 1992
Summary
In Sjogren's syndrome patients, low-grade non-Hodgkin's lymphomas, particularly immunocytomas, are common. These slow-growing lymphomas may even regress spontaneously, suggesting a conservative treatment approach for localized cases.
Area of Science:
- Autoimmune Diseases
- Oncology
- Hematology
Background:
- Sjogren's syndrome is an autoimmune disorder.
- Patients with Sjogren's syndrome have an increased risk of developing lymphoma.
- Understanding lymphoma subtypes and behavior in Sjogren's syndrome is crucial.
Observation:
- A 7-year study at the University of Ioannina followed 120 primary Sjogren's syndrome patients.
- Eight patients (6.7%) developed non-Hodgkin's lymphoma (NHL).
- Lymphomas were diagnosed using the Kiel classification.
Findings:
- The most common lymphomas were low-grade (immunocytoma) (6 cases) and intermediate-grade (2 cases).
- Five immunocytomas occurred in minor salivary or lacrimal glands.
- Monoclonal cell populations were identified via immunoperoxidase staining.
- Two patients experienced spontaneous lymphoma regression, a novel finding in Sjogren's syndrome.
Implications:
- Low-grade NHL, especially immunocytomas, are the predominant lymphomas in Sjogren's syndrome.
- These lymphomas exhibit slow progression and potential for spontaneous regression.
- A conservative treatment strategy is recommended for localized disease in Sjogren's syndrome patients.