Ignite Creation Date:
2024-05-05 @ 11:31 AM
Last Modification Date:
2024-10-26 @ 9:09 AM
Study NCT ID:
NCT00068146
Status:
COMPLETED
Last Update Posted:
2018-07-05
First Post:
2003-09-09
Brief Title:
Fluorodeoxyglucose-Positron Emission Tomography FDG-PET to Evaluate Autoimmune Lymphoproliferative Syndrome ALPS and ALPS-associated Lymphoma
Sponsor:
National Institute of Allergy and Infectious Diseases NIAID
Organization:
National Institutes of Health Clinical Center CC
Study Overview
Official Title:
Study of Fluorodeoxyglucose Positron Emission Tomography FDG-PET for the Evaluation of the Autoimmune Lymphoproliferative Syndrome ALPS and ALPS-Associated Lymphoma
Status:
COMPLETED
Status Verified Date:
2012-11-26
Last Known Status:
None
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
False
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
None
Brief Summary:
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
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
Detailed Description:
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-Hodgkins and Hodgkins 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
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 Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
None
Is a FDA Regulated Device?:
None
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None
Secondary IDs
| Secondary ID | Type | Domain | Link |
|---|---|---|---|
| 02-I-0308 | None | None | None |