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Sinus histiocytosis with massive lymphadenopathy
1Department of Paediatrics and Child Health, University of the Orange Free State, Bloemfontein, South Africa.
Archives of Disease in Childhood
|April 1, 1992
Summary
Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare condition causing enlarged lymph nodes in children. This study describes three pediatric cases, highlighting clinical, laboratory, and biopsy findings for better understanding of this uncommon disease.
Area of Science:
- Pediatric Pathology
- Dermatology
- Oncology
Background:
- Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease, is an uncommon benign proliferation of histiocytes.
- It predominantly affects children and young adults, presenting with painless lymphadenopathy, often cervical.
Purpose of the Study:
- To describe three pediatric cases of Sinus histiocytosis with massive lymphadenopathy presenting with nodal disease.
- To discuss and compare the clinical, laboratory, and biopsy findings with existing literature.
Main Methods:
- Case series review of three pediatric patients diagnosed with SHML.
- Analysis of clinical presentation, laboratory results, and histopathological findings from lymph node biopsies.
- Comparison with previously reported cases in the scientific literature.
Main Results:
- All three cases presented with significant lymphadenopathy.
- Eyelid involvement was noted in one of the described cases.
- Histopathological examination confirmed the diagnosis of SHML, characterized by emperipolesis.
Conclusions:
- Sinus histiocytosis with massive lymphadenopathy is a rare but important differential diagnosis for pediatric lymphadenopathy.
- Comprehensive evaluation including clinical, laboratory, and biopsy data is crucial for accurate diagnosis and management.
- Further research can elucidate the pathogenesis and optimal treatment strategies for SHML.