Status:

COMPLETED

Clinical and Pathophysiological Investigations Into Erdheim Chester Disease

Lead Sponsor:

National Human Genome Research Institute (NHGRI)

Conditions:

Myelofibrosis

Gaucher Disease

Eligibility:

All Genders

2-80 years

Brief Summary

Background: \- Erdheim Chester Disease (ECD) is a very rare disease in which abnormal white blood cells start growing and affect the bones, kidneys, skin, and brain. ECD can cause severe lung disease...

Detailed Description

Rare and potentially lethal, Erdheim-Chester Disease (ECD) is a histiocytic neoplasm about which little is known, and for which there remains a paucity of effective treatments. Histologically, it is c...

Eligibility Criteria

Inclusion

  • INCLUSION CRITERIA:
  • ECD patients of any gender and ethnicity age 2-80 years are eligible to enroll in this protocol.
  • Patients will be diagnosed as having ECD based upon pathologic evaluations of affected organs.
  • Any child older than 2 years with confirmed ECD by pathology will be enrolled in our study since childhood cases are so rare. The child must be clinically stable prior to visiting the NIHCC.
  • Patients that agree to be part of this research study are expected to undergo the evaluations and testing of this protocol.
  • EXCLUSION CRITERIA:
  • Patients will be excluded if they have a suspected diagnosis of ECD not confirmed by biopsy or another form of histiocytosis.
  • -If the patients cannot travel to the NIH because of their medical condition, they can still participate in our study by submitting tissue and blood for research purposes after consent has been obtained and diagnosis of ECD has been confirmed through tissue-biopsy evaluation. This exception applies to all patients aged 2-80 years.
  • Children under age two years are excluded because there is no urgency for a very early diagnosis and care is more readily provided to older children at the Clinical Center.
  • Pregnant women will not take part in this study because of the fetal risks, unless they will be participating by only submitting samples of previously collected tissue. We will not encourage pregnant women to undergo affected organ surgery or biopsy to participate in this study, unless clinically indicated and recommended by their private physicians.

Exclusion

    Key Trial Info

    Start Date :

    August 1 2011

    Trial Type :

    OBSERVATIONAL

    Allocation :

    ACTUAL

    End Date :

    July 24 2019

    Estimated Enrollment :

    88 Patients enrolled

    Trial Details

    Trial ID

    NCT01417520

    Start Date

    August 1 2011

    End Date

    July 24 2019

    Last Update

    July 26 2019

    Active Locations (1)

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    Page 1 of 1 (1 locations)

    1

    National Institutes of Health Clinical Center, 9000 Rockville Pike

    Bethesda, Maryland, United States, 20892