Lymphoproliferative DisordersNCT00068146
Fluorodeoxyglucose-Positron Emission Tomography (FDG-PET) to Evaluate Autoimmune Lymphoproliferative Syndrome (ALPS) and ALPS-associated Lymphoma
An observational study of Lymphoproliferative Disorders and Lymphoma, run by National Institute of Allergy and Infectious Diseases (NIAID) at 1 site in United States.
Trial 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. Read more about this study
- Who is it for?
- Ages 8 years and older
- Conditions the study is for
- Lymphoproliferative Disorders, Lymphoma
- How many people
- 77 participants
- Where
- 1 siteUnited States
- Study dates
- 19 Sep 2002to 26 Nov 2012
- Phase
- Observational
- Are there results?
- None posted in the registry
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 8 years and older
- All sexes
- No healthy volunteers
- Child, Adult, Older adult
Who may be able to join
- ALPS Patients without Lymphoma:
- Participants must:
- Fulfill current criteria for the diagnosis of ALPS as follows:
- Documented chronic nonmalignant lymphadenopathy and/or splenomegaly, and
- Greater than or equal to 1.5 percent TCR alpha/beta+ CD4- CD8- T cells in the peripheral blood.
- Be enrolled in ALPS Natural History Protocol 93-I-0063.
Show all 16Show fewer
- Have clinical evidence of lymphadenopathy as defined by multiple palpable lymph nodes of at least 1cm or radiographic evidence of lymphadenopathy as defined by multiple lymph nodes of at least 1 cm on CT scan, during an evaluation at the NIH Clinical Center.
- Be 8 years of age or older. The study will be targeted to children 8 years of age or older and adults because younger children may not be able to stay still for the duration of the FDG-PET scan procedure. Sedation will not be used in children in this study, except for clinically indicated procedures such as FDG-PET scans in children with lymphoma.
- Be due for their routine [every 2 years] CT scan under protocol 93-I-0063, or be in need of a CT scan for medical reasons [e.g. marked change in adenopathy]. CT scan within 3 months prior to FDG-PET scan is necessary to locate the nodes for appropriate FDG-PET analysis.
- ALPS Patients with Lymphoma:
- Patients must:
- Fulfill current criteria for the diagnosis of ALPS as follows:
- Documented chronic nonmalignant lymphadenopathy and/or splenomegaly.
- Greater than or equal to 1.5 percent TCR alpha/beta + CD4- CD8 -T cells in the peripheral blood.
- Be enrolled in ALPS Natural History Protocol 93-I-0063.
- Have a histologically proven diagnosis of lymphoma, confirmed by the Laboratory of Pathology, NCI (Anatomic Pathology Dept, CC) whether yet treated or not. Lymphomas will be classified according to the WHO classification, using appropriate immunophenotypic and histological features.
Who cannot join
- Patient will be excluded if any of the following are present:
- Concurrent infection or inflammatory disease (e.g., sarcoidosis), which itself often shows increased FDG uptake by PET and which could interfere with the interpretation of study results.
- Active neoplasia other than lymphoma.
- History of chemotherapy or radiation treated malignancy within 5 years prior to study procedure, except for lymphoma.
- Hyperglycermia (regardless of etiology) determined by fasting glucose of greater than 130 mg/dl. Individual with an underlying defect of glucose metabolism may exhibit abnormal metabolism of FDG.
- Weights in excess of 136 kg, which will exceed the weight limit for the scanner table.
- Pregnancy and breast-feeding. For women of childbearing potential, a negative urine or serum pregnancy test is required within 24 hours prior to an FDG-PET scan.
Trial locations and contacts
1 site in 1 country.
United States1 site
National Institutes of Health Clinical Center, 9000 Rockville Pike
Bethesda, Maryland, 20892
About this study
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.
Read more
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.
Read the 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-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.
- Conditions
- Lymphoproliferative Disorders, Lymphoma
- Keywords
- Adenopathy, Genetic Disorder, Apoptosis, Diagnostic Imaging, Hodgkins, Autoimmune Lymphoproliferative, Syndrome, ALPS, ALPS-Associated Lymphoma
- Medical subject headings
- Lymphoproliferative Disorders, Lymphoma, Lymphadenopathy, Genetic Diseases, Inborn, Syndrome
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted9 Sep 2003 (estimated)
- Study start19 Sep 2002
- Study completion26 Nov 2012
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (3)Papers the sponsor has linked to this study.
- Other trials for Lymphoproliferative DisordersStudies of the same condition, open and under way.
- More trials run by National Institute of Allergy and Infectious Diseases (NIAID)The sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Enrolment
- 77 participants (actual)
Sponsor and investigators
- Sponsor type
- US National Institutes of Health
- Investigators
- V. Koneti Rao, M.D., Principal investigator, National Institute of Allergy and Infectious Diseases (NIAID)
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT00068146
- Sponsor's protocol ID
- 020308
- Other IDs
- 02-I-0308
- Status last verified
- 26 Nov 2012
- First submitted
- 9 Sep 2003
- Last updated
- 5 Jul 2018
- Results posted
- No
Publications
Sneller MC, Wang J, Dale JK, Strober W, Middelton LA, Choi Y, Fleisher TA, Lim MS, Jaffe ES, Puck JM, Lenardo MJ, Straus SE. Clincal, immunologic, and genetic features of an autoimmune lymphoproliferative syndrome associated with abnormal lymphocyte apoptosis. Blood. 1997 Feb 15;89(4):1341-8.
PubMed 9028957Straus SE, Sneller M, Lenardo MJ, Puck JM, Strober W. An inherited disorder of lymphocyte apoptosis: the autoimmune lymphoproliferative syndrome. Ann Intern Med. 1999 Apr 6;130(7):591-601. doi: 10.7326/0003-4819-130-7-199904060-00020.
PubMed 10189330Avila NA, Dwyer AJ, Dale JK, Lopatin UA, Sneller MC, Jaffe ES, Puck JM, Straus SE. Autoimmune lymphoproliferative syndrome: a syndrome associated with inherited genetic defects that impair lymphocytic apoptosis--CT and US features. Radiology. 1999 Jul;212(1):257-63. doi: 10.1148/radiology.212.1.r99jl40257.
PubMed 10405750
Study at a glance
From the registry record, last updated by the sponsor on 5 Jul 2018. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Study of Fluorodeoxyglucose Positron Emission Tomography (FDG-PET) for the Evaluation of the Autoimmune Lymphoproliferative Syndrome (ALPS) and ALPS-Associated Lymphoma
- ClinicalTrials.gov ID
- NCT00068146
- Acronym / sponsor ID
- 020308
- Conditions
- Lymphoproliferative Disorders, Lymphoma
- Keywords
- Adenopathy, Genetic Disorder, Apoptosis, Diagnostic Imaging, Hodgkins, Autoimmune Lymphoproliferative, Syndrome, ALPS
- Study type
- Observational
- Recruitment status
- Completed
- Enrolment
- 77 participants (actual)
- Lead sponsor
- National Institute of Allergy and Infectious Diseases (NIAID)US National Institutes of Health
- Study start
- 19 Sep 2002
- Study completion
- 26 Nov 2012
- First posted
- 9 Sep 2003 (estimated)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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.
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-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
Who may qualify
- Ages
- 8 years and older
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Child, Adult, Older adult
Key inclusion criteria (16 listed)
- Fulfill current criteria for the diagnosis of ALPS as follows:
- Be enrolled in ALPS Natural History Protocol 93-I-0063.
- Have clinical evidence of lymphadenopathy as defined by multiple palpable lymph nodes of at least 1cm or radiographic evidence of lymphadenopathy as defined by multiple lymph nodes of at least 1 cm on CT scan, during an evaluation at the NIH Clinical Center.
- Be 8 years of age or older. The study will be targeted to children 8 years of age or older and adults because younger children may not be able to stay still for the duration of the FDG-PET scan procedure. Sedation will not be used in children in this study, except for clinically indicated procedures such as FDG-PET scans in children with lymphoma.
- Be due for their routine [every 2 years] CT scan under protocol 93-I-0063, or be in need of a CT scan for medical reasons [e.g. marked change in adenopathy]. CT scan within 3 months prior to FDG-PET scan is necessary to locate the nodes for appropriate FDG-PET analysis.
Key exclusion criteria (7 listed)
- Patient will be excluded if any of the following are present:
- Concurrent infection or inflammatory disease (e.g., sarcoidosis), which itself often shows increased FDG uptake by PET and which could interfere with the interpretation of study results.
- Active neoplasia other than lymphoma.
- History of chemotherapy or radiation treated malignancy within 5 years prior to study procedure, except for lymphoma.
- Hyperglycermia (regardless of etiology) determined by fasting glucose of greater than 130 mg/dl. Individual with an underlying defect of glucose metabolism may exhibit abnormal metabolism of FDG.
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
Not reported in the source record.
Results and safety
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 5 Jul 2018.
- Background: PubMed 9028957Sneller MC, Wang J, Dale JK, Strober W, Middelton LA, Choi Y, Fleisher TA, Lim MS, Jaffe ES, Puck JM, Lenardo MJ, Straus SE. Clincal, immunologic, and genetic features of an autoimmune lymphoproliferative syndrome associated with abnormal lymphocyte apoptosis. Blood. 1997 Feb 15;89(4):1341-8.
- Background: PubMed 10189330Straus SE, Sneller M, Lenardo MJ, Puck JM, Strober W. An inherited disorder of lymphocyte apoptosis: the autoimmune lymphoproliferative syndrome. Ann Intern Med. 1999 Apr 6;130(7):591-601. doi: 10.7326/0003-4819-130-7-199904060-00020.
- Background: PubMed 10405750Avila NA, Dwyer AJ, Dale JK, Lopatin UA, Sneller MC, Jaffe ES, Puck JM, Straus SE. Autoimmune lymphoproliferative syndrome: a syndrome associated with inherited genetic defects that impair lymphocytic apoptosis--CT and US features. Radiology. 1999 Jul;212(1):257-63. doi: 10.1148/radiology.212.1.r99jl40257.
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
V. Koneti Rao, M.D. principal investigator
National Institute of Allergy and Infectious Diseases (NIAID)
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Lymphoproliferative Disorders and Lymphoma, run by National Institute of Allergy and Infectious Diseases (NIAID) at 1 site in United States.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 16 inclusion and 7 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: finished, as of the sponsor's last update on 5 Jul 2018. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in United States. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT00068146, which also lists 3 linked publications.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 5 Jul 2018.