Jornal de pesquisa de sangue e doenças hematológicas

Autoimmune Diseases And Rosai- Dorfman Disease Coexist More Commonly Than Expected

Lia NL, Asad SD, Jafri SI and Harrison JS

The educational objective herein is to describe 2 case reports in which patients were found to have an autoimmune disease concomitantly with a rare, benign histiocytic disorder known as Rosai-Dorfman disease (RDD). It is unclear if there is an underlying association between autoimmune disease and RDD, as the pathophysiology of RDD has not been well defined to date. Regardless, autoimmune diseases are relatively common in the general population; hence RDD should remain within the differential diagnosis when lymphadenopathy is seen. Lymphadenopathy, although most frequently seen bilaterally in the cervical region in RDD, may present anywhere. A biopsy with histologic confirmation is required to not only evaluate for malignancy in these cases but also necessary to diagnose RDD. Further basic scientific and clinical research is required to fully understand all the characteristics and optimal management of RDD disease. However, we have observed in these two cases presented, that if the autoimmune disease is well controlled, RDD can be an indolent disease.