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Rosai Dorfman disease.
Erum Mazhar1, Naila Rahman, Hina Qureshi
1Department of Hematology, Liaquat National Hospital, Karachi. erumaaz@hotmail.com
Summary
We describe a rare case of Rosai Dorfman disease, a condition causing enlarged lymph nodes. This child experienced eye swelling and neck lymphadenopathy, with a partial response to steroids and subsequent relapse.
Area of Science:
- Pediatric Oncology
- Immunology
- Pathology
Background:
- Sinus histiocytosis with massive lymphadenopathy (SHML), or Rosai Dorfman disease (RDD), is a rare benign proliferative disorder.
- RDD typically presents with painless lymphadenopathy, though extranodal involvement can occur.
Observation:
- This report details an atypical RDD presentation in a pediatric patient.
- The child exhibited bilateral proptosis (bulging eyes) and significant cervical lymphadenopathy.
Findings:
- The patient demonstrated a limited response to initial steroid therapy.
- Disease relapse occurred during the management course, indicating treatment challenges.
Implications:
- This case highlights the variable clinical manifestations of RDD, even in pediatric populations.
- It underscores the need for considering RDD in children with unusual presentations of lymphadenopathy and proptosis.
- Further research into optimal management strategies for relapsing RDD is warranted.