Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease): A Case Report and Literature Review

Abstract

Introduction Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder of unknown etiology. Usually it presents with massive painless cervical lymph node enlargement. Histologically, it shows proliferation of distinctive histiocytic cells that demonstrate emperipolesis in the background of a mixed inflammatory infiltrates. Immunohistochemically, the cells are positive for markers such as CD68 and S100. Objective To report a case of a 12-year-old patient with multiple sites of cervical lymphadenitis, which was diagnosed as RDD histopathologically as well as immunohistologically. Resumed Report A 12-year-old girl presented with multiple painless sites of cervical lymphadenitis without any systemic and other ear, nose, and throat manifestations. The biopsy report of the lymph node showed dilatation of the sinuses, filled with histiocytes having foamy cytoplasm. Many of the histiocytes were engulfing mature lymphocytes. The sinus histiocytes were strongly positive for S-100 protein. Conclusion RDD must be considered in the differential diagnosis of massive or multiple lymphadenopathies.

Long-Lasting Follow-Up with Low-Dose Steroid in an 18-Year-Old Male with Rosai–Dorfman Disease

“…Rosai et al described the clinical manifestations of RDD over detailed analysis of 34 cases in 1972 [1,6,9]. ey reported the first and second decades as the ages of onset [1,10], the male sex was dominant, and SHML was associated to lower socioeconomic status [1]. In general, the disease affects children and adolescents [3,4,7,9,10], but it may also occur in older adults [4]; male and female proportions may vary [4,7,9], and different races are affected equally [9].…”

“…In patients with RDD, bilateral and painless cervical lymphadenopathy is the most prominent clinical manifestation [1][2][3][4][5][9][10][11][12]. Axillary, inguinal, mediastinal, and retroperitoneal lymphadenopathy has been recorded to a minor degree [1,2,4].…”

“…Extranodal involvement, such as the skin [1,[3][4][5]11], scalp, thyroid gland [12], palatine tonsil, soft tissues of the orbit, eyelid, and testicle [1,3], rhinopharynx [9], bone and breast, central nervous system, and gastrointestinal tract, has been reported in patients with RDD [1,3,4]. Even though presentation with fever, night sweats, malaise, and weight loss is common in patients with RDD, they maintain a good general condition [1,3,4,10]. Anemia, leukocytosis with neutrophilia, elevated erythrocyte sedimentation rate, and hyperglobulinemia are the dominating laboratory features [1, 3-5, 7, 9, 13].…”

“…In the literature, there are different modalities recommended for the treatment of RDD [13]. Observation is the preferred choice of therapy in asymptomatic patients or if there is spontaneous resolution of adenopathies [10]. Reports have shown that treatment with steroids resolve fever and reduce lymph node size [1,9,10,13], and the period to resolve clinical symptoms with corticosteroids ranges from 5 days to 6 months [13].…”

“…Observation is the preferred choice of therapy in asymptomatic patients or if there is spontaneous resolution of adenopathies [10]. Reports have shown that treatment with steroids resolve fever and reduce lymph node size [1,9,10,13], and the period to resolve clinical symptoms with corticosteroids ranges from 5 days to 6 months [13]. In our case, after 38 months of oral corticosteroid treatment, the symptoms disappeared completely, the spleen returned to its normal size, but the lymph nodes showed slight regression following disease diagnosis.…”