Journal of Postgraduate Medicine
 Open access journal indexed with Index Medicus & ISI's SCI  
Users online: 6146  
Home | Subscribe | Feedback | Login 
About Latest Articles Back-Issues Articlesmenu-bullet Search Instructions Online Submission Subscribe Etcetera Contact
 ::  Similar in PUBMED
 ::  Search Pubmed for
 ::  Search in Google Scholar for
 ::  Article in PDF (946 KB)
 ::  Citation Manager
 ::  Access Statistics
 ::  Reader Comments
 ::  Email Alert *
 ::  Add to My List *
* Registration required (free) 

  IN THIS Article
 ::  References
 ::  Article Figures

 Article Access Statistics
    PDF Downloaded16    
    Comments [Add]    
    Cited by others 3    

Recommend this journal


  Table of Contents     
Year : 2019  |  Volume : 65  |  Issue : 1  |  Page : 62-63

Rosai Dorfman disease and peripheral T-cell lymphoma: A rare co-occurrence

Department of Radiation Oncology, Gandhi Medical College, Bhopal, Madhya Pradesh, India

Date of Web Publication28-Jan-2019

Correspondence Address:
Dr. V Tiwari
Department of Radiation Oncology, Gandhi Medical College, Bhopal, Madhya Pradesh
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpgm.JPGM_356_18

Rights and Permissions

How to cite this article:
Tiwari V, Pareek A, Ghori H, Ahirwar M. Rosai Dorfman disease and peripheral T-cell lymphoma: A rare co-occurrence. J Postgrad Med 2019;65:62-3

How to cite this URL:
Tiwari V, Pareek A, Ghori H, Ahirwar M. Rosai Dorfman disease and peripheral T-cell lymphoma: A rare co-occurrence. J Postgrad Med [serial online] 2019 [cited 2023 Jun 7];65:62-3. Available from:

Rosai–Dorfman disease (RDD) is a rare nonmalignant condition described as a separate clinico-pathological entity by Rosai and Dorfman in 1969. RDD belongs to a group of nonmalignant histiocytic disorders characterized by an increase in the number of histiocytes (mainly mononuclear phagocytic cells and antigen presenting cells) of bone marrow origin in nodal or extranodal sites.[1] Histologically, there is presence of pericapsular fibrosis and dilated sinuses, heavy infiltrated large histiocytes, lymphocytes, and plasma cells in the lymph nodes (LNs). The presence of emperipolesis in histiocytes is considered diagnostic of RDD.[2] In this communication, we report a case of RDD occurring concurrently with peripheral T-cell variant of Non-Hodgkin's lymphoma (NHL) emphasizing its rarity.

A 60- year-old female presented with bilateral multiple painless neck swellings and associated fever of four months duration. On examination, multiple swellings of various sizes were palpable at left parotid, neck nodal levels Ia, Ib, II, III, IV, and level V bilaterally [Figure 1]a. The swellings were nontender, firm, and mobile. Multiple LNs were also palpable at bilateral axillary regions along with a right epitrochlear LN. Fine needle aspiration cytology revealed increased prominence of sinuses, histocytic proliferation, and histiocytes containing macrophages (emperipolesis) that mandated immunohistochemistry (IHC) correlation to exclude RDD [Figure 1]b. Subsequent biopsy showed complete effacement of LN architecture by diffuse infiltration of monomorphic atypical lymphoid cells. These cells were having features favoring NHL. The IHC staining revealed positive S100, positive CD68 staining of histiocytes, and absent CD1a features consistent with RDD along with positive leukocyte common antigen, CD3 and negative CD20, epithelial membrane antigen, Tdt and MIC-2, and favoring the diagnosis of a peripheral T cell NHL [Figure 2]. Patient was planned for six cycles of cyclophosphamide, hydroxydaunorubicin (also called doxorubicin), oncovin (vincristine) and prednisone regimen q21 day's cycle (injection cyclophosphamide 900 mg I/V, injection adriamycin 50 mg I/V, injection vincristine 1.6 mg I/V, and tab prednisolone 100 mg po). Patient received six cycles of chemotherapy, and the treatment period was uneventful. Patient is on regular follow-up, and clinically, there is no evidence of disease [Figure 1]c.
Figure 1: (a) Pre-treatment clinical picture showing bilateral neck lymphadenopathy,(b) Fine needle aspiration cytology image H and E, ×40 showing macrophages with prominent emperiopolesis (arrow) , and (c) Post-treatment clinical picture showing complete resolution of neck lymphadenopathy

Click here to view
Figure 2: Immunohistochemistry stain positivity (a) CD 68 (b) Diffuse CD3 (c) Diffuse LCA, and (d) S100

Click here to view

Although etiology of RDD is still precarious, infectious (Varicella Zoster, Epstein–Barr virus, Cytomegalovirus,  Brucella More Details, Klebsiella), genetic, and inflammatory processes have been postulated in its pathogenesis.[3] There is an increase in the number of histiocytes, mainly mononuclear phagocytic cells and antigen presenting cells of bone marrow origin in nodal or extranodal sites.[1] Microscopically, LN shows presence of pericapsular fibrosis and dilated sinuses, heavily infiltrated with large histiocytes, lymphocytes, and plasma cells. The presence of emperipolesis that stains positive for S100, and CD 68 is considered diagnostic for RDD.[2] Apart from S100 and CD68, IHC stains are positive for CD163, α1-antichymotrypsin, α1-antitrypisn, fascin, and HAM-56 and are typically negative for CD1a.[2] Most commonly, patients present with bilateral massive painless lymphadenopathy with fever, night sweats, and weight loss.

The co-occurrence of RDD with lymphoma (Hodgkin's or NHL) has been rarely reported.[4] On extensive search of literature, we found 20 cases of RDD occurring concomitantly with lymphoma (Hodgkin's or NHL).[5] In majority of the cases, RDD precedes malignant lymphoma, and in very few cases, RDD occurs synchronously with NHL.[5]

The clinical course of RDD is unpredictable; it can remain indolent for years and sometimes regresses spontaneously. Rarely does it manifest any systemic symptoms or organ dysfunction, usually sparing the bone marrow, spleen, and liver.[4] There is controversy over the treatment protocol and usually “wait and watch” approach is acceptable for most patients. The available treatment options include steroid therapy, surgery, or single agent cytotoxic therapy.[6]

While diagnosing RDD, clinicians should maintain a high suspicion for the presence of occult lymphoma. We recommend a holistic approach in patients diagnosed with RDD to exclude the presence of occult lymphoma.

Declaration of patient consent

The authors certify that appropriate patient consent was obtained.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

 :: References Top

Henter JI, Tondini C, Pritchard J. Histiocyte disorders. Crit Rev Oncol Hematol 2004;50:157-74.  Back to cited text no. 1
Juskevicius R, Finlay JL. Rosai-Dorfman: Disease of the parotid gland, cytologic and histopathologic findings with immunohistochemical correlation. Arch Pathol Lab Med 2001;125:1348-50.  Back to cited text no. 2
Levine PH, Jahan N, Murari P, Manak M, Jaffe ES. Detection of human herpesvirus 6 in tissues involved by sinus histiocytosis with massive lymphadenopathy (Rosai- Dorfman Disease). J Infect Dis 1992;166:291-5.  Back to cited text no. 3
Melikyan AL, Kovrigina AM, Gilyazitdinova EA, Gitis MK. A case of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) in a patient with diffuse large B-cell lymphoma and chronic hepatitis B virus infection. Ter Arkh 2012;84:66-70.  Back to cited text no. 4
Moore JC, Zhao X, Nelson EL. Concomitant: Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman Disease) and diffuse large B-cell lymphoma: A case report. J Med Case Rep 2008;2:70.  Back to cited text no. 5
Oka M, Kamo T, Goto N, Nagano T, Hirayama Y, Nibu K, et al. Successful treatment of Rosai-Dorfman disease with low-dose oral corticosteroid. J Dermatol 2009;36:237-40.  Back to cited text no. 6


  [Figure 1], [Figure 2]

This article has been cited by
1 How I Diagnose Rosai-Dorfman Disease
Aishwarya Ravindran, Karen L Rech
American Journal of Clinical Pathology. 2023;
[Pubmed] | [DOI]
2 Strong Coexpression of Transcription Factors PU.1 and Oct-2 in Rosai-Dorfman Disease
Kala Gnanasekaran Kiruthiga, Sheren Younes, Yasodha Natkunam
American Journal of Clinical Pathology. 2022;
[Pubmed] | [DOI]
3 Rosai-Dorfman disease: an overview
Cassandra Bruce-Brand, Johann W Schneider, Pawel Schubert
Journal of Clinical Pathology. 2020; 73(11): 697
[Pubmed] | [DOI]


Print this article  Email this article
Online since 12th February '04
2004 - Journal of Postgraduate Medicine
Official Publication of the Staff Society of the Seth GS Medical College and KEM Hospital, Mumbai, India
Published by Wolters Kluwer - Medknow