Cytomorphology and Histology Correlation of Rosai-Dorfman Disease: A 15-Year Study from a Tertiary Referral Centre in South India

Abstract

Objectives: Rosai-Dorfman disease (RDD) is an uncommon, benign histiocytic disorder of unknown etiology, typically presenting in young adulthood. We highlight the cytomorphology of RDD and correlate it with the histopathology. Study Design: All cases diagnosed as RDD on fine-needle aspiration cytology between January 2001 and June 2015 were included. Clinical details were obtained from medical records. The cytology smears were reviewed along with the histopathology and immunohistochemistry, wherever available. Results: The study included 10 cases ranging in age from 11 to 68 years (median 29). There was a male predominance with a male:female ratio of 1.5:1. The patients commonly presented with bilateral cervical lymphadenopathy. Extranodal involvement was seen in 2 cases in the nose and mandible, respectively. Of these 10 cases, 8 were later biopsied. The cytological features included numerous crescentic histiocytes, emperipolesis, reactive lymphocytes and plasma cells. A histological diagnosis of RDD was made in 7 out of 8 cases, and 1 was diagnosed as Hodgkin lymphoma. Conclusion: FNA represents an efficient, minimally invasive, cost-effective and reliable technique for the diagnosis of RDD and may obviate the need for further biopsy. However, the disease has close differential diagnoses, including Langerhans cell histiocytosis, granulomatous lesions, and Hodgkin lymphoma. Hence, it must be remembered that there can be pitfalls when the diagnosis is made by cytology alone.

A Rare Case of Rosai-Dorfman disease presented as generalized lymphadenopathy: A case Report

“…[5] FNA may be su cient to make the diagnosis in most cases thus preventing unnecessary invasive procedures and helps to rule out common clinical differential diagnosis . [6, 7,19] Histologically, there is an in ltration of the tissue by lymphocytes, histiocytes, and plasma cells. The presence of emperipolesis (histiocytes with engulfed lymphocytes, erythrocytes, and plasma cells) is usually characteristic of Rosai-Dorfman-Destombes disease along with dilated sinusoids .…”

“…[31] The differential diagnoses can include reactive lymph node hyperplasia, infectious lymphadenitis, Langerhans cell histiocytosis, non-Hodgkin's lymphoma, and metastatic carcinoma. [11,19] The rarity of RDD has limited our understanding of the choice of the best treatment. [2] Therapy is unnecessary in most cases.…”

“…[13] The clinical ndings in such cases can include painless enlargement of the cervical lymph nodes, fever, leukocytosis, anemia, hypergammaglobulinemia, and elevated erythrocyte sedimentation rate (ESR). [19] Rapid on-site evaluation (ROSE) with ne-needle aspiration cytology (FNAC) is a cost-effective, rapid method that can be used for cytological diagnosis of RDD.…”

Rosai-Dorfman disease with renal involvement and associated autoimmune haemolytic anaemia in a 12-year-old girl: A case report

“…It has a prevalence of 1 in 200,000 [2]. It commonly occurs in the second and third decades of life [3,4]. Pathophysiology and natural history remain concealed due to the low prevalence of disease.…”