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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy, SHML) is a rare, non-hereditary, benign histiocytic proliferative disorder mainly affecting young people with common clinical characteristics such as painless bilateral cervical lymphadenopathy, fever, leukocytosis and polyclonal gammopathy. Extranodal manifestations have been reported in 28-43% of cases. Eye involvement is relatively uncommon (8.5%), and most of cases have presented as lymphoproliferation in the soft tissues of the orbit and eyelids. Uveitis is an even more rare presentation as a review of all the literature. We describe a 63-year-old man with SHML with unusual ophthalmic manifestations of relapsing uveitis and bilateral subconjunctival masses. The results of biopsies were compatible with the characteristic histopathological findings of SHML: focal aggregations of S100-positive foamy histiocytes and the existence of lymphocytophagocytosis. During the clinical course, the patient relapsed but the relapse was relatively benign and the patient showed fair response to topical as well as systemic corticosteroid treatment. Here we describe this unusual presentation of SHML to inform physicians of the possibility for this systemic granulomatous disease to contribute to relapsing uveitis.
Chang Gung Med J 2002 Sep
PMID:Rosai-Dorfman disease manifesting as relapsing uveitis and subconjunctival masses. 1247 25

Sinus histiocytosis with massive lymphadenopathy is a rare histiocytic disorder with very few case reports in Indian literature. Immunological abnormalities have been documented in few cases. We report one such case of a child presenting with generalized lymphadenopathy and complicated by autoimmune hemolytic anemia, suggestive of an associated immune dysfunction.
Indian J Pediatr 2003 Sep
PMID:Sinus histiocytosis with massive lymphadenopathy: complicated by autoimmune hemolytic anemia. 1462 Jan 95

A case of extranodal sinus histiocytosis with massive lymphadenopathy (ENSHML; Rosai-Dorfman disease) is reported. The patient presented with a history of intracranial tumour and exophthalmus. Clinical examination found a large mass in the left orbit and paranasal sinuses. Excisional biopsy showed a dense fibrous tissue with an infiltrate rich in macrophages. Further evaluation revealed a retroperitoneal mass with consecutive ureteral stenosis. Further histological and immunohistochemical investigation of the orbital mass, now in suspicion of a systemic disease showed an infiltrate of S-100-positive histiocytes and emperipolesis allowing the diagnosis of extranodal sinus histiocytosis. The correct histologic diagnosis was delayed due to the unusual and isolated extranodal localisation of the disease. The literature concerning extranodal manifestations of Rosai-Dorfman disease is reviewed. We suggest the additional evaluation of such rare and unusual cases in experienced reference centers.
Pathologe 2004 Sep
PMID:[Isolated Rosai-Dorfman disease]. 1512 28

Rosai-Dorfman disease (RDD) is a systemic disease and its etiology is not well understood. It is a very rare but established disease. We report a case of RDD diagnosed in the upper airway that became a life-threatening condition because of the stricture of the subglottic space. A 49-year-old female complained of nasal obstruction, hoarseness and lacrimation. After evaluation following by fiberscopic examination and CT scanning of the head and neck, sinusitis and laryngeal masses were detected. Endoscopic sinus surgery and endolaryngeal microsurgery were performed simultaneously. The masses in the nasal cavity and larynx showed the same histological findings. Proliferative large histiocytes with voluminous clear cytoplasm and rounded nuclei, and lymphophagocytosis known as emperipolesis are the characteristic features of RDD. In this case, extranodal manifestations in the subglottic space gradually emerged postoperatively. Tracheotomy was performed to ensure an airway and prevent suffocation. An extensive treatment is not required in most patients with RDD, however, some patients who have extranodal manifestations involving the airway may detoriorate into a life-threatening condition.
Auris Nasus Larynx 2004 Sep
PMID:Rosai-Dorfman disease revealed in the upper airway: a case report and review of the literature. 1536 64

Sinus histiocytosis with massive lymphadenopathy (SHML or Rosai-Dorfman disease) is a nonneoplastic, usually self-limiting disease. Alhough it affects all age groups, it is more commonly seen in young males in their first or second decades. The disease primarily manifests as painless lymphadenopathy of cervical region, but other nodal groups and extranodal sites may also be affected. Seldom SHML may involve the salivary glands. We present the cytologic features and differential diagnoses of one such case.A 45-yr-old woman presented with an enlarged submandibular gland on the left side with ipsilateral cervical lymphadenopathy that had been persisting for 1 mo. The gland and the two enlarged nodes measured 2.5 x 2.5 cm(2) each and were firm in consistency. Clinically, tumor of the salivary glands was suspected. Fine-needle aspiration (FNA) smears showed moderate cellularity, with large histiocytes dispersed in the background of intense lymphoplasmacytic infiltrate. These histiocytes showed lymphophagocytosis. Isolated stromal fragments consisting of fibrocytes were seen separate from salivary acinar clusters on repeat aspirations. The diagnosis of SHML involving left submandibular gland and ipsilateral lymph nodes was returned. When analyzed in the context of clinical findings (laboratory data), the cytologic features of SHML involving salivary gland could be differentiated from those of malignancies and other benign lesions, especially Kuttner's tumor of the submandibular gland, which mimics neoplasm clinically.
Diagn Cytopathol 2005 Sep
PMID:Salivary gland manifestations of sinus histiocytosis with massive lymphadenopathy: fine-needle aspiration cytology findings. A case report. 1607 53

Rosai-Dorfman disease (RDD), originally described as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. Patients usually present with massive cervical lymphadenopathy. However other nodal sites (mediastinal and inguinal) are also frequently involved. The retro-peritoneum is an infrequent site of involvement. This uncommon entity should also be included in the list of differential diagnoses of diffuse and massive lymphadenopathy, which includes includes infectious/granulomatous conditions, lymphomatous and metastatic disease.
Br J Radiol 2005 Sep
PMID:Rosai-Dorfman disease: unusual cause of diffuse and massive retroperitoneal lymphadenopathy. 1611 Jan 9

Rosai-Dorfman disease is a rare, idiopathic, benign, histiocytic proliferation usually seen in younger patients. Most commonly neck lymph nodes are involved with a predominant infiltration of sinusoidal histiocytes. Nearly half of the patients have extranodal manifestation. Two cases of Rosai-Dorfman disease involving the nose and the paranasal sinuses are presented. The clinical presentation, radiographic findings, treatment and histological characteristics of the disease are discussed.
Rhinology 2005 Sep
PMID:Nasal and paranasal sinus manifestation of Rosai-Dorfman disease. 1621 19

We report a case of extranodal Rosai-Dorfman disease (RDD) (sinus histiocytosis with massive lymphadenopathy) presenting with a solitary active lesion of the femur.
Radiat Med 2005 Sep
PMID:Extranodal Rosai-Dorfman disease: a solitary lesion with soft tissue reaction. 1638 88

Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease, is a very rare disorder of unknown etiology. Although classical SHML usually presents as massive, painless, bilateral neck lymph node enlargement, approximately 25% to 40% of SHML cases described to date present in extranodal organs or tissues, such as skin, upper respiratory tract, and bone etc. However, bone marrow as a site of initial presentation by SHML has not been described since the disease was initially recognized in 1969. Here, we report the first case of bone marrow involvement by extranodal SHML, which occured in a 73 year-old man with a history of refractory idiopathic thrombocytopenia purpura. The bone marrow biopsy demonstrates the distinctive and characteristic morphologic and immunophenotypic features of extranodal SHML.
Am J Surg Pathol 2006 Sep
PMID:Extranodal Rosai-Dorfman disease involving the bone marrow: a case report. 1693 65

Rosai-Dorfman disease is a rare, histiocytic proliferative disorder of unknown etiology commonly affecting lymph nodes. Extranodal lesions with or without nodal involvement also occur. We report the case of a 63 year-old woman with disseminated Rosai-Dorfman disease involving the neurohypophysis and associated with adenohypophysial PRL cell hyperplasia.
Pituitary 2010 Sep
PMID:Rosai-Dorfman disease involving the neurohypophysis. 2040 24


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