Rosai-Dorfman Disease: A previously unreported association with Sickle Cell Disease

Stebbing, Claire; Walt, Jon van der; Ramadan, G.; Inusa, Baba

doi:10.1186/1472-6890-7-3

Cited by 8 publications

(7 citation statements)

References 3 publications

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“…This implies that a person's genotype may not play a contributory role. However, our patient and the patient of Stebbing et al [11] had been transfused in the past. Could there be a possible association of RDD with blood transfusion?…”

Section: Case Reportmentioning

confidence: 63%

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Rosai-Dorfman disease in a 12-year-old Nigerian male

2016

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Section: Case Reportmentioning

confidence: 63%

“…[2] Stebbing et al [11] reported a possible association with sickle cell disease. The haemoglobin electrophoresis of our patient was AA.…”

Section: Case Reportmentioning

confidence: 99%

Rosai-Dorfman disease in a 12-year-old Nigerian male

2016

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“…Yoon and colleagues had theorized that the initiation of monocyte colony-stimulating factor-mediated histoproliferation in RDD is an abnormal reaction of the hematolymphoid system to infection, leading to a high level of immune activation and subsequent cytosis [5]. However, the pathogenesis of RDD is still poorly understood but is likely multifactorial, with many of the documented patients having a variety of coexisting immunologically mediated disorders such as asthma, systemic lupus erythematosus, rheumatoid arthritis and hemolytic anemia [2,21]. …”

Section: Discussionmentioning

confidence: 99%

Extranodal Rosai–Dorfman disease: a rare soft tissue neoplasm masquerading as a sarcoma

et al. 2013

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IntroductionRosai–Dorfman disease (RDD) is a rare proliferative histiocytic disorder of unknown etiology. RDD typically presents with generalized lymphadenopathy and polymorphic histiocytic infiltration of the lymph node sinuses; however, occurrences of extranodal soft tissue RDD may rarely occur when masquerading as a soft tissue sarcoma.Materials and methodsA comprehensive search of all published cases of soft tissue RDD without associated lymphadenopathy was conducted using PubMed and Google Scholar for the years 1988 to 2011. Ophthalmic RDD was excluded.ResultsThirty-six cases of extranodal soft tissue RDD, including the current one, have been reported since 1988. Anatomical distribution varied among patients. Four (11.1%) patients presented with bilateral lesions in the same anatomic region. Pain was the most common symptom in six (16.8%) patients. Sixteen (41.6%) patients were managed surgically, of which one (2.8%) case experienced recurrence of disease.ConclusionRDD is a rare inflammatory non-neoplastic process that should be considered in the differential diagnosis of a soft tissue tumor. Thus, differentiation of extranodal RDD from more common soft tissue tumors such as soft tissue sarcoma or inflammatory myofibroblastic tumor is often difficult and typically requires definitive surgical excision with histopathological examination. While the optimal treatment for extranodal RDD remains ill-defined and controversial, surgical excision is typically curative.

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“…SHML may closely simulate other disease states in its clinical manifestations, especially malignant lymphoma, histiocytosis and non-neoplastic disorders including storage diseases. RDD is occasionally complicated by sickle cell disease, autoimmune lymphoproliferative syndrome and histiocytic sarcoma in the literature 4 5…”

Section: Discussionmentioning

confidence: 99%

Unusual life-threatening Rosai-Dorfman disease of the trachea: role of NF- B

Zhou

Chen

Zhu

et al. 2010

Thorax

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RosaieDorfman disease (RDD) is a rare non-neoplastic histioproliferative disorder characterised by painless lymphadenopathy, low fever, high erythrocyte sedimentation rate, leucocytosis and hypergammaglobulinaemia. Overactivity of nuclear factor kB (NF-kB) is linked with inflammatory, cancerous and autoimmune diseases. The first case is described of an unusual life-threatening RDD of the trachea with no lymphadenopathy at risk of suffocation in a 39-year-old Chinese woman. A diagnosis of RDD was made following CT scans, thoracotomy and histological examination. Gel shift assay revealed an essential role for NF-kB overactivity in RDD. The patient remains well with no evidence of progression without treatment. Histological confirmation should be sought in all cases as the clinical manifestation of RDD is similar to asthma or lung carcinoma.Sinus histiocytosis with massive lymphadenopathy (SHML) is a syndrome first recognised by Rosai and Dorfman in 1969. 1 As a rare non-neoplastic histiocytic proliferative disorder, RosaieDorfman disease (RDD) is characterised by bilateral painless cervical lymphadenopathy as well as low fever, high erythrocyte sedimentation rate, leucocytosis and hypergammaglobulinaemia. Extranodal involvement including the skin, orbit and nasopharynx occurs in approximately one-third of patients. 2Overactivity of nuclear factor kB p50/p65) is linked with inflammatory, cancerous and autoimmune diseases. 3 We describe an uncommon case of life-threatening RDD of the trachea in association with NF-kB overactivity.

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Rosai-Dorfman Disease: A previously unreported association with Sickle Cell Disease

Abstract: Background: Rosai-Dorfman Disease is an uncommon benign systemic histio-proliferative disease. This is the first time the disease, although more common in people of African descent, is described in association with Sickle cell disease.

Cited by 8 publications

References 3 publications

Rosai-Dorfman disease in a 12-year-old Nigerian male

Rosai-Dorfman disease in a 12-year-old Nigerian male

Extranodal Rosai–Dorfman disease: a rare soft tissue neoplasm masquerading as a sarcoma

Unusual life-threatening Rosai-Dorfman disease of the trachea: role of NF- B

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