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Top 10 Facts about Rosai Dorfman Disease 

Dr Sampurna Roy MD   

 

 

 

What is Rosai Dorfman Disease [Pathology Infographic]

 

(1)  History:

Rosai-Dorfman disease (RDD), originally known as sinus histiocytosis with massive lymphadenopathy, is a non-Langerhans cell histiocytosis that was first described in 1965 by Destombes and subsequently recognized as a distinct entity by Rosai and Dorfman in 1969.

 

(2) Presentation

Patients with this rare and nonmalignant disorder often present with painless cervical lymphadenopathy, fever, and leukocytosis.

Usually affects healthy young adults and adolescents.

 

(3)  Extranodal sites

RDD often manifests in the lymph nodes of the head and neck, but it may also present in extranodal sites, skin, respiratory tract, bone, genitourinary system, oral cavity, central nervous system, eyes/orbit/ocular adnexa, salivary gland, tonsil, breast, soft tissue and heart.

The bone marrow is rarely involved. 

 

(4)  Etiology

The etiology of RDD is unknown though it has been speculated that  chronic infection or an immune response to an infectious agent or an antigen causing the initial histiocytic proliferation.

 

(5)  Microscopic features:

The classic histology is characterized by effacement of nodal architecture and dilatation of lymph node sinuses by lymphocytes, plasma cells and numerous characteristic histiocytes with large vesicular nuclei and abundant clear cytoplasm.

 

(6)   Emperipolesis

Many of these histiocytes, also known as RDD cells, contain intact lymphocytes, and sometimes plasma cells, neutrophils and red blood cells, within their cytoplasm.

This process whereby cells enter and transit through a cell evading cellular degradation is known as emperipolesis and was first described by Humble et al.

When extranodal sites are involved, similar morphologic features to the nodal counterpart are seen although with more fibrosis, fewer typical RDD histiocytes, and less prominent emperipolesis.

 

(7)  Immunohistochemical stains :

Immunohistochemical stains are useful when diagnosing RDD as the RDD cells have been found to express pan-macrophage antigens (CD68, HAM56, CD14, etc.), antigens associated with phagocytosis (CD64, Fc receptor for immunoglobulin G), lysosomal activity (lysozyme alpha 1-antitrypsin, alpha1-antichymotrypsin), and immune activation and adhesion molecules (transferring receptor, interleukin 2 receptor). 

The most consistent and reliable phenotype for RDD is S100(+), CD68(+) and CD1a(−).

 

(8)   Cutaneous Rosai-Dorfman disease

The term (cutaneous Rosai-Dorfman disease) CRDD is used to describe the forms of RDD that only involve the skin.

It presents as erythematous to brown papules, plaques, or nodules.

CRDD differs from RDD in that RDD exhibits systemic involvement.

Unlike RDD, more commonly in young adult males, CRDD  typically occurs in older females. 

There is no predilection for site and patients with CRDD are free from systemic manifestations .

Approximately 10% of RDD patients exhibit skin lesions, and in 3% of cases, the disease presents exclusively in the skin .

Purely CRDD was reported for the first time by Thawerani et al  in 1978 in a 48-year-old male patient who presented with a solitary nodule on the shoulder.

Histological findings in CRDD are usually similar to those in RDD.

Typically, the epidermis is normal.

In the dermis, a diffuse infiltrate of histiocytes is accompanied by a background infiltrate of lymphocytes and plasma cells.

Lymphoid follicles with reactive germinal centers may also be present.

The phenomenon of emperipolesis, which represents the presence of intact lymphocytes within histiocytes, is common in CRDD.

Less often, the cytoplasm may contain plasma cells, neutrophils and red blood cells.

Mitoses and nuclear atypia are rare, while necrosis is absent.

CRDD histiocytes stain positively for S100 protein and CD68, but negatively for CD1a, which can be used to confirm the diagnosis of CRDD.

Electronic microscopy of CRDD tissue reveals no signs of Birbeck granules, which eliminates the possibility of Langerhans cell histiocytosis.

 

(9)  Differential diagnoses:

The differential diagnoses of RDD-type lesions include lymphoreticular malignancies when cervical lymphadenopathy is present or soft tissue sarcomas when patients present with extranodal disease. 

A lack of cytologic atypia typically dispels more malignant diagnoses, and immunohistochemistry profiles will demonstrate a macrophage-induced histiocytosis with emperipolesis.

It is also important to clearly distinguish RDD-similar S-100-positive histiocytoses such as malignant histiocytosis and LCH.

Malignant histiocytosis demonstrates marked cytologic atypia as well as high mitotic activity, while LCH tends to be CD1a positive with microscopic evidence of Birbeck granules.

In addition, neither malignant histiocytosis nor LCH demonstrate the hallmark finding of emperipolesis.

The more rare reticulohistiocytoma may be S-100-positive; however, this disease shows prominent ground glass appearance, abundant periodic acid Schiff-positive stain and fewer inflammatory cells in the background.

These marker-specific differences are useful in providing a definitive diagnosis.

 

(10)  Prognosis:

Most patients with RDD have a complete and spontaneous remission.

Some may experience recurrent or persistent but stable lymphadenopathy.

In rare cases, the disease follows an aggressive course and may be fatal.

Involvement of kidney, lower respiratory tract, or liver has been found to be a poor prognostic sign.

 

Source:

 

Shi Y, Griffin AC, Zhang PJ, Palmer JN, Gupta P. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman Disease): A case report and review of 49 cases with fine needle aspiration cytology. CytoJournal. 2011;8:3. 

 

FANG S, CHEN A-J. Facial cutaneous Rosai-Dorfman disease: A case report and literature review. Experimental and Therapeutic Medicine. 2015;9(4):1389-1392. 

 

Komaragiri M, Sparber LS, Santos-Zabala ML, Dardik M, Chamberlain RS. Extranodal Rosai–Dorfman disease: a rare soft tissue neoplasm masquerading as a sarcoma. World Journal of Surgical Oncology. 2013;11:63. 

 

 

 

 

 

 

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