Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): Report of two cases with fine-needle aspiration cytology

• Published in: Diagnostic cytopathology Vol. 24; no. 1; pp. 42 - 45
• Main Authors: Das, Dilip K., Gulati, Achal, Bhatt, Naveen C., Sethi, Gulshan R.
• Format: Journal Article
• Language: English
• Published: New York John Wiley & Sons, Inc 01.01.2001; Wiley-Liss
• Subjects: Adolescent; Biological and medical sciences; Biopsy, Needle; Child, Preschool; emperipolesis; Female; Glucocorticoids - therapeutic use; Hematologic and hematopoietic diseases; Histiocytosis, Sinus - complications; Histiocytosis, Sinus - drug therapy; Histiocytosis, Sinus - pathology; Humans; Lymph Nodes - pathology; lymphophagocytosis; Medical sciences; Other diseases. Hematologic involvement in other diseases; Rosai-Dorfman disease; SHML; Tropical medicine; Human; Immunohistochemistry; Hemopathy; Aspiration punction; Case study; Pathology; Fine needle; Sinus histiocytosis; Adenopathy; Adolescent; Rosai-Dorfman disease; Cytopathology; Female; Child
• ISSN: 8755-1039; 1097-0339
• DOI: 10.1002/1097-0339(200101)24:1<42::AID-DC1007>3.0.CO;2-N

Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai‐Dorfman disease is a rare but well‐defined histiocytic proliferative disorder. There are only few reports or small series of cases on the fine‐needle aspiration (FNA) cytologic features of this entity. Our first case was a 14‐yr‐old girl who presented with low‐grade fever and neck swellings of 2 mo duration. FNA smears from the submandibular and cervical lymph nodes showed numerous histiocytes with evidence of lymphophagocytosis (emperipolesis) against a background of reactive lymphoid cells. The FNA cytodiagnosis was Rosai‐Dorfman disease. The patient was put on steroids, with improvement. The second patient was a 3½‐yr‐old girl who presented with fever and left cervical lymphadenopathy. The initial FNA smear from an outside laboratory was reported as reactive hyperplasia. A repeat FNA smear showed numerous histiocytes with evidence of emperipolesis, mild nuclear enlargement and multilobation/multinucleation in a few histiocytes, and reactive lymphoid cells. The cytodiagnosis was Rosai‐Dorfman disease. Review of the initial smear showed the Rosai‐Dorfman (RD) cells, but in smaller number. We conclude that FNA cytology is a useful tool in the diagnosis of SHML, but the diagnosis may be missed at initial stages when characteristic cytomorphologic features are not well‐developed. Diagn. Cytopathol. 2001;24:42–45. © 2001 Wiley‐Liss, Inc.