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Sinus Histiocytosis (Rosai-Dorfman Disease)

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Benign, non-Langerhans-cell, histiocytic proliferative disorder that primarily affects the lymph nodes. It is often referred to as sinus histiocytosis with massive lymphadenopathy.

Also Known As:

Rosai-Dorfman Disease; Histiocytosis, Sinus; Sinus Histiocytosis with Massive Lymphadenopathy; Destombes Rosai Dorfman Syndrome; Disease, Rosai-Dorfman; Histiocytoses, Sinus; Rosai Dorfman Disease; Sinus Histiocytoses; Syndrome, Destombes-Rosai-Dorfman; Destombes-Rosai-Dorfman Syndrome

Networked: 185 relevant articles (12 outcomes, 6 trials/studies)

Relationship Network

Disease Context: Research Results

Hemic and Lymphatic Diseases
- Lymphatic Diseases: 2658
  - Histiocytosis: 321
    - Non-Langerhans-Cell Histiocytosis: 35
      - Sinus Histiocytosis: 185
        Asrar Facharzt Haque syndrome

Related Diseases

1.	Histiocytic Necrotizing Lymphadenitis (Kikuchi Disease)
2.	Hodgkin Disease (Hodgkin's Disease)
3.	Paraparesis
4.	Non-Langerhans-Cell Histiocytosis (Histiocytosis, Non Langerhans Cell)
5.	Optic Nerve Diseases (Optic Neuropathy)

Experts

1.	Kumar, Rakesh: 2 articles (01/2016 - 03/2014)
2.	Zhou, Tao: 2 articles (10/2015 - 03/2005)
3.	Wang, Fang: 2 articles (04/2015 - 04/2014)
4.	O'Malley, Dennis P: 2 articles (05/2013 - 12/2010)
5.	Bechtel, Mark: 2 articles (08/2012 - 09/2010)
6.	Darabi, Kamruz: 2 articles (08/2012 - 09/2010)
7.	Peters, Sara: 2 articles (08/2012 - 09/2010)
8.	Hornick, Jason L: 2 articles (11/2011 - 11/2007)
9.	Warnke, Roger A: 2 articles (02/2010 - 05/2005)
10.	Dalence, Carlos: 2 articles (02/2010 - 10/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Sinus Histiocytosis:

1.	Adrenal Cortex Hormones (Corticosteroids)IBA 03/01/2014 - "The diagnosis of Rosai-Dorfman disease is usually good, since 40 % of the patients present a spontaneous remission if they are treated with oral corticosteroids, even though the lesion can be managed with fractionated radiotherapy or with radical surgery. " 07/01/2012 - "We describe a case of Rosai-Dorfman disease that failed to respond to monotherapy with systemic corticosteroids but improved with the addition of methotrexate. " 07/01/2012 - "The rapid improvement of clinical and physical symptoms with minimal side effects in our case and others reinforces the utility of antimetabolites in patients with Rosai-Dorfman disease who fail to respond to corticosteroids." 06/24/2015 - "Cutaneous Rosai-Dorfman Disease Located on the Breast: Rapid Effectiveness of Methotrexate After Failure of Topical Corticosteroids, Acitretin and Thalidomide."
2.	Methotrexate (Mexate)FDA LinkGeneric 07/01/2014 - "Cutaneous Rosai-Dorfman disease successfully treated with low-dose methotrexate." 07/01/2012 - "To review the classic physical and histologic findings in Rosai-Dorfman disease and summarize the literature supporting the use of methotrexate in this condition. " 07/01/2005 - "Venorelbine and methotrexate for the treatment of Rosai-Dorfman disease." 07/01/2012 - "We describe a case of Rosai-Dorfman disease that failed to respond to monotherapy with systemic corticosteroids but improved with the addition of methotrexate. " 07/01/2012 - "Rosai-Dorfman disease treated with methotrexate and low-dose prednisone: case report and review of the literature."
3.	Thalidomide (Thalomid)FDA Link 05/01/2003 - "Cutaneous Rosai-Dorfman disease: remission with thalidomide treatment." 04/01/2014 - "Dermatoscopic findings in cutaneous Rosai-Dorfman disease and response to low-dose thalidomide." 08/01/2013 - "Successful treatment of Rosai-Dorfman disease with low-dose oral thalidomide." 06/24/2015 - "Cutaneous Rosai-Dorfman Disease Located on the Breast: Rapid Effectiveness of Methotrexate After Failure of Topical Corticosteroids, Acitretin and Thalidomide."
4.	Acitretin FDA Link 09/01/2010 - "The objective of this report is to illustrate that acitretin may be effective in treating cutaneous Rosai-Dorfman disease. " 09/01/2010 - "Our experience with a patient with a case of cutaneous Rosai-Dorfman disease persisting after surgical excision demonstrates that acitretin may be an effective therapeutic option for cutaneous Rosai-Dorfman disease." 09/01/2010 - "Cutaneous rosai-dorfman disease persisting after surgical excision: report of a case treated with acitretin." 11/01/2007 - "Extensive purely cutaneous Rosai-Dorfman disease responsive to acitretin." 09/01/2010 - "A patient with cutaneous Rosai-Dorfman disease refractory to antibiotics, steroids, and surgical excision was treated with acitretin for a period of four months and was closely monitored for improvement in the lesion. "
5.	Muramidase (Lysozyme)IBA 10/01/1999 - "Immunophenotypic studies, which showed a CD68+, lysozyme+, UCHL-1+, OPD4-, CD4-, s100- profile, were helpful in distinguishing these lesions from other processes, including Kikuchi-Fujimoto disease and Rosai-Dorfman disease. " 09/01/1987 - "Sinus cells in ordinary sinus histiocytosis were constantly positive for ACT and variably positive for lysozyme and were generally negative for S-100 protein. " 01/01/2002 - "Positivity for S-100 protein, alpha1-antitrypsin, alpha1-antichymotrypsin, lysozyme, Mac387 and CD68 proteins, and negativity for CD1a protein confirmed the diagnosis of Rosai-Dorfman disease. "
6.	Cladribine FDA LinkGeneric 12/01/2006 - "Dramatic clinical efficacy of cladribine in Rosai-Dorfman disease and evolution of the cytokine profile: towards a new therapeutic approach." 01/01/2009 - "Extranodal multifocal Rosai-Dorfman disease: response to 2-chlorodeoxyadenosine treatment." 10/15/2006 - "Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) treated with 2-chlorodeoxyadenosine." 01/01/2014 - "A single case of rosai-dorfman disease marked by pathologic fractures, kidney failure, and liver cirrhosis treated with single-agent cladribine."
7.	Interferon-alpha (Interferon Alfa)FDA Link 06/02/2001 - "[Long term survival in a patient with aggressive Rosai-Dorfman disease treated with interferon alpha]." 05/01/1997 - "Sinus histiocytosis with massive lymphadenopathy: complete response to low-dose interferon-alpha." 12/01/1995 - "Long-term survival in a patient with Rosai-Dorfman disease treated with interferon-alpha." 02/03/1996 - "[Sinus histiocytosis of the Rosai-Dorfman type following Hodgkin disease: efficacy of interferon-alpha]."
8.	SteroidsIBA 11/01/2011 - "Symptomatic extranodal Rosai-Dorfman disease treated with steroids, radiation, and surgery." 09/01/2003 - "Response of cutaneous Rosai-Dorfman disease to topical and intralesional steroids." 09/01/2010 - "A patient with cutaneous Rosai-Dorfman disease refractory to antibiotics, steroids, and surgical excision was treated with acitretin for a period of four months and was closely monitored for improvement in the lesion. "
9.	Anti-Bacterial Agents (Antibiotics)IBA 09/01/2010 - "A patient with cutaneous Rosai-Dorfman disease refractory to antibiotics, steroids, and surgical excision was treated with acitretin for a period of four months and was closely monitored for improvement in the lesion. "
10.	imatinib (Gleevec)FDA Link 05/01/2009 - "A case of cutaneous Rosai-Dorfman disease refractory to imatinib therapy." 05/01/2009 - "Rosai-Dorfman disease is a non-Langerhans cell histiocytosis that recently has been treated successfully with imatinib mesylate in a patient with a systemic variant of the disease. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 01/01/1983 - "Sinus histiocytosis with massive lymphadenopathy: remission with chemotherapy." 01/01/2015 - "Histopathology of the lesion was reported as being consistent with Rosai-Dorfman disease and the patient is currently undergoing chemotherapy. " 05/01/2006 - "Each patient with Rosai-Dorfman disease had unique disease expression requiring aggressive therapy, such as chemotherapy, radiation, and/or surgical excision. " 01/01/1995 - "Evidence of responsiveness to chemotherapy in aggressive Rosai-Dorfman disease." 12/01/1998 - "This case demonstrates the potential efficacy of chemotherapy in the treatment of compressive optic neuropathy in cases of orbital sinus histiocytosis with massive lymphadenopathy."
2.	Radiotherapy 10/27/2014 - "Extranodal rosai-dorfman disease of the bone treated with surgery and radiotherapy." 11/01/2012 - "Cutaneous Rosai-Dorfman disease of the right ear responsive to radiotherapy." 06/01/2011 - "Radiotherapy for steroid-resistant laryngeal Rosai-Dorfman disease." 10/01/2008 - "A case of orbital Rosai-Dorfman disease responding to radiotherapy." 10/01/2001 - "Review of the literature on intracranial Rosai-Dorfman disease leads to the suggestion that postoperative radiotherapy may be advisable in all cases."
3.	Radical Mastectomy 08/01/1985 - "[Sinus histiocytosis in the lymph nodes of breast cancer patients having undergone extensive radical mastectomy]." 05/01/1991 - "A prospective study of comparable Japanese and British breast cancer patients treated by radical mastectomy confirmed previously reported findings that sinus histiocytosis and germinal center hyperplasia are more frequently seen in axillary lymph nodes from Japanese than in those from British patients. " 11/02/1974 - "The degree of lymphocytic infiltration in and around breast tumours together with sinus histiocytosis and follicular hyperplasia in regional lymph nodes has been studied in 310 cases of breast cancer treated with standard radical mastectomy. "
4.	Joint Prosthesis 10/01/1995 - "Sinus histiocytosis mimicking metastatic melanoma in lymph nodes of a patient with a large joint prosthesis: case report and review of the literature." 12/01/1989 - "Sinus histiocytosis seen in the lymph nodes draining the sites of joint prostheses may resemble, and must be distinguished from, other conditions invoking a sinus pattern of lymphadenopathy, as well from benign and malignant diseases that involve the lymph nodes in a pattern mimicking sinus histiocytosis." 10/01/1995 - "Malignant melanoma metastases to regional lymph nodes may be mimicked by several non-neoplastic processes, including sinus histiocytosis induced by fragments shed from joint prostheses. "
5.	Transplants (Transplant) 02/01/2012 - "Reconstruction of a patient with Rosai-Dorfman disease using ramus graft and osseointegrated implants: a case report."