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Rosai-Dorfman Disease
B N Mahanta1, T Goswami Mahanta2
1Associate Professor Medicine.
Insights
Rosai Dorfman Disease, a rare condition, caused recurrent neck swellings in a young male. The disease was diagnosed via fine-needle aspiration cytology and immunohistochemistry, and the patient improved with supportive care.
Area of Science:
- Medical Pathology
- Immunohistochemistry
- Rare Diseases
Background:
- Recurrent neck swellings can indicate various underlying pathologies.
- Accurate diagnosis is crucial for effective patient management.
Observation:
- A young male presented with recurrent cervical lymphadenopathy.
- Initial investigations revealed elevated leukocyte count and non-caseating granulomas on ultrasound-guided biopsy.
- Fine-needle aspiration cytology (FNAC) showed characteristic histiocytic and lymphophagocytic features.
Findings:
- Immunohistochemistry confirmed histiocytic markers (CD68) and proliferation index (Ki-67).
- CD25 expression was also noted on histiocytes.
- The findings were consistent with a diagnosis of Rosai-Dorfman Disease.
Implications:
- This case highlights the diagnostic utility of FNAC and immunohistochemistry in Rosai-Dorfman Disease.
- Supportive treatment alone may be sufficient for managing this rare condition.
- Further research into optimal therapeutic strategies for Rosai-Dorfman Disease is warranted.
Abstract:
A young male presented with recurrent neck swellings with initial leucocyte count of 16800/mm3, with non-caseating glands on ultrasonography neck. FNAC showed mixed cellularity with histiocytic and marked lymphophagocytosis as seen in Rosai Dorfman Disease. Immunohistochemistry demonstrated CD 25, Ki 67 and CD 68 in histiocytes. Her responded to supportive treatment alone.
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