Enamul Karim

Rosai Dorfman Disease

It is a very rare disorder. Less than 1000 cases reported in literature1. RDD originally described as Sinus Histiocytosis with Massive Lymphadenopathy, presents in its most typical form as massive painless bilateral cervical lymphnode enlargement in the neck, associated with fever, leukocytosis, elevated ESR and polyclonal Hypergammaglobulinaemia. It may occur at any age but mostly seen in young adults and children with aslight predilection for males (58%) and for inviduals of African descent2,3. Although an etiology is unknown, the disease is thought to be a disorder of immune regulation or response to a presumed infectious agents (HHV-6/EBV) with its major manifestation in lymphnodes with resultant proliferation of sinusoidal histiocytes4. Most efforts to identify a pathogen has culminated in indeterminate or negative results5,6. Approximately 25% to 40% of SHML cases described to date present in extra nodal organs or tissues , the most common are eye and ocular adnexa(esp. Orbit), head neck region6, upper respiratory tract, skin and subcutaneous tissue4,5, skeletal system and CNS[2,3]. Organs that are almost always spared are lung, spleen , bone marrow(the latter exclusive of focal involvement)7,8. Diseases that may be associated with RDD are amyloidosis, AIHA, HIV, Lymphoma etc9-11. ROSAI DORFMAN DISEASE