A case of Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Syndrome) from Western India

INTRODUCTION Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman Syndrome, a nonmalignant variant of histiocytosis (1) is a chronic reactive disorder. It presents as a benign, chronic, massive enlargement of cervical lymph nodes (LN) accompanied by fever, leukocytosis, elevated erythrocyte sedimentation rate (ESR) and hyperglobulinamia (2). There is no malignant proliferation of the class II histiocytes (i.e. normal macrophages and monocytes). The etiology of this disease is currently unclear. Sinus histiocytosis with massive lymphadenopathy may be of two types – either familial or infection induced (2). With respect to the latter, increased antibody titers to Epstein Barr and measles viruses have been observed; however, etiological evidence is lacking (1). Immune disturbances are likely to be a feature in some patients. Subtle undefined immunological defects are also considered as a causal factor. Human Herpes Virus6 DNA has been detected in biopsy specimens and is considered as a contributing factor (6). Sinuous histiocytosis with massive lymphadenopathy (SHML) most frequently occurs in first and second decades of life, with a peak incidence at twenty years of age (1). There is a higher prevalence in males, and among Afro-Caribbeans as compared to Caucasians and Asians. Also, a familial association has been observed in some cases (3).