This is also known as sinus histiocytosis with massive lymphadenopathy. It is a rare and benign disorder. The etiology of this disease is not known. It is characterized by formation of increased production of histiocytes which accumulate in lymph nodes throughout the body.

 

Lypmhnodes of neck are the common place of histiocytes accumulation. Other than lypmhnodes the other sites involved are skin, upper respiratory tract and bone. It is also known as Destombes-Rosai-Dorfman syndrome. It is often confused with lymphoma.

Signs and symptoms

• Massive painless lymphadenopathy in neck bilaterally
• Fever
• Weight loss
• Tonsillitis
• Nasal discharge
• Nasal obstruction
• Liver enlargement
• Spleen enlargement

Diagnosis:

ESR: Increased

Lypmhnodes biopsy with histopathological examination shows dilated sinuses containing histiocytes.

Treatment:

• Surgery
• Chemotherapy with vinca alkaloid combined with an alkylating agent and corticosteroid.
• Radiation therapy.