YO13 - Rosai-Dorfman Disease Presenting with Multiple Contiguous Lymphadenopathy: A Case Report

Case summary

Sinus histiocytosis with massive lymphadenopathy (SHML) also known as Rosai-Dorfman disease (RDD), is a rare entity of lymph nodes and was first described by Rosai and Dorfman in 1969. The disease affects predominantly the younger age group. Painless lymphadenopathy is the most frequent presenting symptoms and involved the cervical lymph node in up to 90% of patients, which is typically bilateral and self-limiting. It is a benign proliferative histiocytic disorder morphologically characterized by ‘emperipolesis’.

We report a case of a 47-year-old man with multiple lymphadenopathies involving the cervical, axillary, intra-abdominal, and inguinal region suggestive of lymphoma. Histopathological and immunohistochemistry studies of a cervical and inguinal lymph node biopsy established our diagnosis of Rosai-Dorfman disease. Our patient did not have evidence of airway compromise, extranodal involvement and distant metastasis. Hence, watch and wait approach was adopted.

Episodes of remission and exacerbation may occur for several years. In approximately 70% of cases the disease is permanent but stable, 20% experience spontaneous and permanent remission and 10% suffer from progressive and generalized disease.

Multidisciplinary collaboration is often vital to the evaluation and management of patients with RDD and systematic investigation of novel therapies for RDD is needed. Comprehensive RDD evaluation involves careful medical history, physical examination, imaging studies, and laboratory evaluations to determine the extent of disease and presence of co-occurring disorders.

In conclusion, RDD is a rare, inflammatory nonneoplastic process which most often decreases in size without aggressive management. As RDD is infrequently suspected clinically and is a rare disease process, an awareness of the disease entity along with its clinical profile and its consideration in the differential diagnosis in the evaluation of histiocytic and lymphocytic pathologies is essential for proper interpretation and diagnosis and management of this disease.

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