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 ?
Background: Rosai-Dorfman disease (RDD) is typically characterized by painless bilateral and symmetrical cervical lymphadenopathy, with associated fever and leukocytosis.
plasma cells, (27,34) including granulomatosis with polyangiitis, (35) multicentric Castleman disease, (36) Rosai-Dorfman disease, (37) marginal zone lymphoma, (38-41) pancreatic adenocarcinoma, (42,43) and even reactive lymphadenopathy, (44) to name just a few.
Q Our granddaughter has been diagnosed with Rosai-Dorfman disease (RD).
Rosai-Dorfman disease (RDD), otherwise known as sinus histiocytosis with massive lymphadenopathy (SHML), is a rare and benign histiocytic proliferative disorder of unknown aetiology.
A single case of Rosai-Dorfman disease marked by pathological fractures, kidney failure, and liver cirrhosis treated by a single agent cladribine.
The bilateral tibiae uptake was very inhomogeneous, lytic areas on the right anterior tuberosity and left diaphysis suggesting a possible association with LCH or Rosai-Dorfman Disease (RDD) [2, 8].
Rosai-Dorfman disease (RDD), also known as "sinus histiocytosis with massive lymphadenopathy," is a nonmalignant disease of histiocyte proliferation.
DISCUSSION: Rosai-Dorfman disease was first described as a distinct clinic-pathologic entity by Rosai and Dorfman in 1969.
Rosai-Dorfman disease is a non-neoplastic disease of unknown etiology observed mainly in children and adolescents affecting mainly the lymph nodes.
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.
1 Rosai-Dorfman disease (RDD) and Juvenile Xantho-granuloma (JXG) are two different histiocytosis that share several characteristics including their benign course.