Central China Rosai-Dorfman Disease Registry

Langerhans Cell Histiocytosis Clinical Trial

— CCR-DDR  

Official title:

A Trial to Evaluate the Long Term Prognosis in Rosai-Dorfman Disease

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT05284942
• Other study ID #: Tongji0509
• Status: Recruiting
• Phase: Phase 4
• Start date: October 1, 2011
• Est. completion date: June 1, 2022

Study information

• Verified date: March 2022
• Source: Tongji Hospital
• Contact: YIKAI YU, M.D
• Phone: +1-484-995-5917
• Email: yuyikai0509[@]gmail.com
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial Summary

This is a research study among patients with Rosai-Dorfman disease.

Description:

Rosai-Dorfman disease is a rare disorder characterized by overproduction (proliferation) and accumulation of a specific type of white blood cell (histiocyte) in the lymph nodes of the body (lymphadenopathy), most often those of the neck (cervical lymphadenopathy). In some cases, abnormal accumulation of histiocytes may occur in other areas of the body besides the lymph nodes (extranodal). These areas include the skin, central nervous system, kidney, and digestive tract. The symptoms and physical findings associated with Rosai-Dorfman disease vary depending upon the specific areas of the body that are affected. The disorder predominantly affects mainly adolescents or young adults. The exact cause of Rosa Dorfman disease is unknown. The purpose of this research study is to learn the prevalence , therapies and long term prognosis of Rosa Dorfman disease.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 20
• Est. completion date: June 1, 2022
• Est. primary completion date: June 1, 2022
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years to 80 Years

Eligibility

Inclusion Criteria:

Diagnosis of a histiocytic disorder as determined by a corroborating constellation of histopathology, clinical, and/or radiologic findings. All participants will undergo Standard of Care MRA, CTA or imaging examination. Exclusion Criteria: Known intracranial involvement of histiocytosis (including dura, leptomeninges and brain parenchyma) Prior stroke or intracranial hemorrhage Other (non-histiocytic) intracranial neoplasm or neurological disorder deemed by the PI or Co-PI to confound neuroimaging studies (e.g., demyelinating disease) Existing diagnosis of a psychiatric disorder or untreated mood disturbance Existing diagnosis of a neurodegenerative disease, such as Alzheimer's disease Chronic or daily excessive alcohol consumption as determined by the PI. History of chronic use of corticosteroids, defined as continuous treatment for six months or longer at any time in the past History of severe claustrophobia or other contraindications to patient SOC brain MRI Prior intravenous cytarabine or cladribine Other current or prior treatments (e.g., high-dose chemotherapy for a different cancer) deemed by the PI or Co-PI to confound imaging studies or cognitive performance

Related Conditions & MeSH terms

• Histiocytosis
• Histiocytosis, Langerhans-Cell
• Langerhans Cell Histiocytosis

Intervention

Drug:
• Mycophenolate mofetil combined with oral prednisone
Mycophenolate mofetil oral 500mg twice a day combined with standard of care of oral prednisone 5mg-15mg/day from baseline to week 108

Locations

Country	Name	City	State
China	Tongji Hospital	Wuhan	Hubei
China	Tongji Hospital	Wuhan	Hubei

Sponsors (1)

Lead Sponsor	Collaborator
Tongji Hospital

Country where clinical trial is conducted

China, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Autoimmune bullous disease quality of life (ABQoL)	Total ABQoL scores range from 0 to 30 . The ABQoL score is calculated by summing the score of each question resulting in a maximum of 30 and a minimum of 0. The higher the score, the more quality of life is impaired.	From baseline to 108 Weeks
Secondary	Cumulative Oral Corticosteroid Dose	Calculate the Cumulative Oral Corticosteroid Dose	From 12th, 24th, 36th ,52th and 108 Weeks