Fluorodeoxyglucose-Positron Emission Tomography (FDG-PET) to Evaluate Autoimmune Lymphoproliferative Syndrome (ALPS) and ALPS-associated Lymphoma
|ClinicalTrials.gov Identifier: NCT00068146|
Recruitment Status : Completed
First Posted : September 9, 2003
Last Update Posted : October 6, 2017
|First Submitted Date||September 9, 2003|
|First Posted Date||September 9, 2003|
|Last Update Posted Date||October 6, 2017|
|Start Date||September 19, 2002|
|Primary Completion Date||Not Provided|
|Current Primary Outcome Measures||Not Provided|
|Original Primary Outcome Measures||Not Provided|
|Change History||Complete list of historical versions of study NCT00068146 on ClinicalTrials.gov Archive Site|
|Current Secondary Outcome Measures||Not Provided|
|Original Secondary Outcome Measures||Not Provided|
|Current Other Outcome Measures||Not Provided|
|Original Other Outcome Measures||Not Provided|
|Brief Title||Fluorodeoxyglucose-Positron Emission Tomography (FDG-PET) to Evaluate Autoimmune Lymphoproliferative Syndrome (ALPS) and ALPS-associated Lymphoma|
|Official Title||Study of Fluorodeoxyglucose Positron Emission Tomography (FDG-PET) for the Evaluation of the Autoimmune Lymphoproliferative Syndrome (ALPS) and ALPS-Associated Lymphoma|
This study will evaluate the usefulness of FDG-PET scanning in distinguishing autoimmune lymphoproliferative syndrome (ALPS) from lymphoma. Lymphoma is cancer of the lymph system. ALPS is a condition involving persistent enlargement of the lymph glands, spleen, or liver, and a range of other problems relating to blood cell counts and abnormal immune activity, in which the immune system attacks healthy tissues. People with ALPS particularly those with an abnormal Fas gene also have an increased risk of developing lymphoma. The Fas gene codes for a protein that causes immune cells called lymphocytes to die when they are no longer needed.
FDG-PET is a new nuclear imaging test that is very effective in detecting lymphoma. It is important to identify these cancers as quickly as possible, since some are very curable when caught early. Since ALPS and lymphoma share several common characteristics, a reliable, non-invasive method of distinguishing the two, such as FDG-PET might offer, is crucial. FDG-PET uses a radioactive sugar molecule to produce images that show the metabolic activity of tissues. Because cancer cells grow and divide more rapidly than normal cells, they metabolize more sugar for fuel. This increased activity identifies them as cancer in FDG-PET scanning. For this procedure, the subject is injected with the sugar molecule and lies in a doughnut-shaped machine (PET camera) for the imaging.
Adults and children 10 years old or older with ALPS, with or without lymphoma, may be eligible for this study. Candidates will be screened with a physical examination, blood tests, and computed tomography (CT) scan.
Participants will have an FDG-PET scan and a DEXA scan. The DEXA scan measures fat and non-fat tissue and is used help interpret the FDG-PET results. For this test, the subject lies on a table while a fast X-ray is taken from head to toe.
Patients who develop signs or symptoms suggesting the development or recurrence of lymphoma (such as further enlargement of lymph glands, unexplained fever or weight loss, or abnormal scans) may undergo a tissue biopsy. For this procedure, a small piece of lymph or other tissue is surgically removed for examination under the microscope. In addition, patients who develop these symptoms may be asked to undergo additional FDG-PET scans up to two a year in patients without lymphoma, and as many as needed in patients with lymphoma to evaluate their response to treatment and guide future therapy.
The Autoimmune Lymphoproliferative Syndrome (ALPS) is an inherited disorder associated with defective lymphocyte apoptosis, which is clinically characterized by prominent non-malignant lymphadenopathy, hepatosplenomegaly and overt autoimmune diseases such as hemolytic anemia, autoimmune thrombocytopenia and neutropenia. Additionally, ALPS patients have a significantly increased risk of developing non-Hodgkin's and Hodgkin's lymphoma.
The diagnosis of lymphoma is particularly troublesome in ALPS because many ALPS manifestations overlap with clinical features suggestive of lymphoma. Therefore, individuals with ALPS may undergo repeated biopsies during the course of the disease. Finding a non-invasive test that can predictably discriminate benign from malignant lymphadenopathy in ALPS, and that can help discern whether a more invasive lymph node biopsy is necessary, would be very desirable.
Fluorodeoxyglucose Positron Emission Tomography (FDG-PET) is an increasingly used non-invasive imaging technique for staging and monitoring therapeutic responses in patients with lymphoma. This technique might be able to assist us in distinguishing whether enlargement of lymph nodes is due to ALPS versus ALPS associated lymphoma. However, FDG-PET has not been studied in patients with ALPS. This study will first explore whether ALPS patients with lymphadenopathy show FDG uptake. If uptake is shown, the study will obtain initial quantitative data to compare FDG uptake in ALPS patients with lymphadenopathy, and ALPS patients with associated lymphoma. The ultimate goal is to assess FDG-PET as a reliable non-invasive method to differentiate lymphadenopathy due to ALPS versus that of ALPS associated lymphoma.
|Study Design||Not Provided|
|Target Follow-Up Duration||Not Provided|
|Sampling Method||Not Provided|
|Study Population||Not Provided|
|Study Groups/Cohorts||Not Provided|
* Includes publications given by the data provider as well as publications identified by ClinicalTrials.gov Identifier (NCT Number) in Medline.
|Estimated Completion Date||November 26, 2012|
|Primary Completion Date||Not Provided|
ALPS Patients without Lymphoma:
ALPS Patients with Lymphoma:
|Ages||8 Years and older (Child, Adult, Senior)|
|Accepts Healthy Volunteers||No|
|Contacts||Contact information is only displayed when the study is recruiting subjects|
|Listed Location Countries||United States|
|Removed Location Countries|
|Other Study ID Numbers||020308
|Has Data Monitoring Committee||Not Provided|
|U.S. FDA-regulated Product||Not Provided|
|IPD Sharing Statement||Not Provided|
|Responsible Party||Not Provided|
|Study Sponsor||National Institute of Allergy and Infectious Diseases (NIAID)|
|PRS Account||National Institutes of Health Clinical Center (CC)|
|Verification Date||November 26, 2012|