Rosai-Dorfman disease

si·nus his·ti·o·cy·to·sis with mas·sive lym·phad·e·nop·a·thy

a chronic disease occurring in children and characterized by massive painless cervical lymphadenopathy due to distension of the lymphatic sinuses by macrophages containing ingested lymphocytes, and by capsular and pericapsular fibrosis.

Rosai-Dorfman disease

(rō′zī-dôrf′măn′, -mən)
n.
A chronic disease characterized by painless, massively enlarged cervical lymph nodes whose sinuses contain histiocytes that have engulfed lymphocytes and other cells. Extranodal involvement sometimes occurs.

Rosai-Dorfman disease

A rare, benign idiopathic condition characterised by bilateral cervical lymphadenopathy. It is most common in young black men and women, but may affect other ages and races; it may be extranodal. 

Clinical findings
Massive bilateral cervical lymphadenopathy, fever.

Lab
Leukocytosis, increased erythrocyte sedimentation rate, polyclonal hyperimmunoglobulinaemia.
 
Prognosis
Uncertain; most resolve spontaneously, but some are aggressive and ultimately fatal.

si·nus his·ti·o·cy·to·sis with mas·sive lym·phad·e·nop·a·thy

(sī'nŭs his'tē-ō-sī-tō'sis mas'iv limf-ad-ĕ-nop'ă-thē)
A chronic disease occurring in children characterized by massive painless cervical lymphadenopathy due to distension of the lymphatic sinuses by macrophages containing ingested lymphocytes, and by capsular and pericapsular fibrosis.
Synonym(s): Rosai-Dorfman disease.

Rosai,

Juan, U.S. pathologist, 1941–.
Rosai-Dorfman disease - Synonym(s): sinus histiocytosis with massive lymphadenopathy
References in periodicals archive ?
Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is an uncommon disorder.
The differential diagnosis of JXG should include other histiocytic lesions, Rosai-Dorfman disease, and infectious causes.
Rosai-Dorfman Disease, or Sinus Histiocytosis with Massive Lymphadenopathy (SHML), is a rare benign, self-limited disease with a predilection for head and neck involvement.
Rosai-Dorfman disease is an idiopathic histiocytic proliferative disorder characterized by sinus histiocytosis and massive lymphadenopathy.
The FNAB tests showed numerous lymphocytes and histiocytes with emperipolesis (Figure 3, B); Ziehl-Neelsen stain results for acid-fast bacilli were negative, and the features were considered most consistent with Rosai-Dorfman disease.
Rosai-Dorfman disease is a rare histiocyte disorder that is typically characterized by massive cervical lymphadenopathy.
9) Thus, JXG and Langerhans cell histiocytosis are both categorized as dendritic cell-related disorders, as opposed to macrophage-related proliferative disorders such as Rosai-Dorfman disease.
The histologic differential diagnosis includes lymphoma, osteomyelitis, extranodal Rosai-Dorfman disease, and Erdheim-Chester disease.
Prominent macrophages and microglia seen in the biopsy were not associated with Langerhans histiocytes or emperipolesis, thus excluding Langerhans histiocytosis and Rosai-Dorfman disease.
LCH must be distinguished from reactive histiocytosis, Hodgkin lymphoma, NK/T-cell lymphoma, ErdheimChester disease (a CD 1 a-negative histiocytic disorder), and Rosai-Dorfman disease.
Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is a rare, benign, histiocytic condition of unknown etiology.
Rosai-Dorfman disease in an Asian Indian woman with classic generalized lymphadenopathy and nasal obstruction: A case report