Usefulness of oral corticosteroid in Rosai?Dorfman disease

Ocheni, S; Ibegbulam, Obike; Okafor, Okechukwu Charles; Raveenthiran, V; Nzegwu, M.A.

doi:10.1111/j.1365-2354.2006.00746.x

Cited by 19 publications

(18 citation statements)

References 10 publications

(9 reference statements)

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“…There has been some reported benefit from the use of corticosteroids,9 but the use of chemotherapeutic agents has not proved to be beneficial. The prognosis is variable, with reported deaths10 and also a good outcome given the benign nature of the disease which tends to regress without any intervention.…”

Section: Discussionmentioning

confidence: 99%

Rosai-Dorfman disease of the lung

Ali

Mackay

2009

Thorax

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Rosai-Dorfman disease is a rare disorder which seems to involve not only the lymph nodes but most other organs of the body. The case history is presented of a woman who was thought to have atypical carcinoma of the lung but in whom a diagnosis of Rosai-Dorfman disease was eventually made following a range of investigations including CT scans, positron emission tomography (PET) and histological analysis of open lung biopsy specimens. She remains well with no further progression of the disease without treatment. Rosai-Dorfman disease can mimic lung carcinoma and shows increased activity on PET scanning, so histological confirmation should be sought in all cases.

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Section: Discussionmentioning

confidence: 99%

Rosai-Dorfman disease of the lung

Ali

Mackay

2009

Thorax

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“…Since infectious agents have been suspected in the pathogenesis, antibiotics and acyclovir were used; revealing inconsistent results [17][18][19][20]. Corticosteroids are frequently employed in patients with bulky disease and they appear to control compression symptoms [21]. They may induce remission in some cases but steroid dependence may present a problem as recurrence is frequent when steroids are tapered [22,23].…”

Section: Discussionmentioning

confidence: 99%

Extranodal multifocal Rosai–Dorfman disease: response to 2-chlorodeoxyadenosine treatment

et al. 2008

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Rosai-Dorfman disease (RDD) or "sinus histiocytosis with massive lymphadenopathy" is a rare lymphoproliferative disorder of unknown etiology. The disease usually presents with painless lymphadenopathy with occasional extranodal involvement in various organs. We report a case of a 36-year-old man with a history of non-Hodgkin lymphoma (NHL), who recently presented with inguinal lymphadenopathy. Following the diagnosis of RDD on lymph node biopsy, he developed symptoms of spinal cord compression due to a mass lesion discovered at T6-7 vertebral level. 18F-Fluorodeoxyglucose (18FDG) positron emission tomography (PET-CT) revealed extensive disease with lung, renal and bone involvement. The patient received a short course of steroid therapy for cord compression findings and 2-chlorodeoxyadenosine (2-CdA) treatment was initiated for long-term disease control. He had a dramatic sustained response to treatment with six courses of 2-CdA. These results suggest that 2-CdA can be an effective treatment of choice and positron emission tomography with 18FDG can be used for determining the extent of disease and for follow-up in RDD.

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“…Multiple chemotherapeutic agents have been tried with variable degrees of success and there has been some reported benefit from the use of corticosteroids 16 17Learning points

Rosai-Dorfman disease (RDD) is a rare non-malignant proliferation of histiocytes of unknown aetiology that mainly affects lymph nodes and various extranodal sites as well.

RDD should be considered in the differential diagnosis of interstitial lung disease even in the absence of lymphadenopathy.

There is an overlap between RDD and IgG4 disease.

Emperipolesis is a distinctive feature of RDD.

…”

Section: Discussionmentioning

confidence: 99%

Pulmonary IgG4+ Rosai-Dorfman disease

El‐Kersh

Perez

2013

BMJ Case Reports

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Rosai-Dorfman disease (RDD) is a rare non-malignant proliferation of histiocytes of unknown aetiology that mainly affects lymph nodes. Here we report a case of RDD that presented a diagnostic dilemma due to its atypical presentation and the overlap with IgG4 disease. Our case presented with interstitial lung involvement without lymphadenopathy. Open lung biopsy suggested the diagnosis of RDD. However, the predominant IgG4 positive plasma cells together with the absence of lymphadenopathy were not typical of RDD. Within 1 year, the patient developed diffuse lymphadenopathy and immunohistochemical staining of lymph node aspirates confirmed the diagnosis. Despite trials of corticosteroid therapy, the disease progressed.

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Usefulness of oral corticosteroid in Rosai?Dorfman disease

Cited by 19 publications

References 10 publications

Rosai-Dorfman disease of the lung

Rosai-Dorfman disease of the lung

Extranodal multifocal Rosai–Dorfman disease: response to 2-chlorodeoxyadenosine treatment

Pulmonary IgG4+ Rosai-Dorfman disease

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