Autoimmune Lymphoproliferative SyndromeNCT01672918
Fluorodeoxyglucose Imaging Studies to Detect Lymphoma
An observational study of Autoimmune Lymphoproliferative Syndrome and Lymphoma, run by National Institute of Allergy and Infectious Diseases (NIAID).
Trial summary
Background: Autoimmune lymphoproliferative syndrome (ALPS) is a genetic disorder of the lymph system. People with ALPS often have swollen lymph nodes, especially in the neck and armpit. They also have a much higher risk of developing lymphoma. Read more about this study
- Who is it for?
- Ages 5 to 99 years
- Conditions the study is for
- Autoimmune Lymphoproliferative Syndrome, Lymphoma
- How many people
- 0 participants
- Where
- No sites listed yet
- Study dates
- 6 Aug 2012to 27 Feb 2015
- 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 5 to 99 years
- All sexes
- No healthy volunteers
- Child, Adult, Older adult
Who may be able to join
- To qualify for enrollment, patients must meet all of the following:
- Fulfill current criteria for the diagnosis of ALPS, which includes documented chronic nonmalignant lymphadenopathy and/or splenomegaly, and either greater than or equal to 1.5% T-cell receptor alpha/beta+ DNTs in the peripheral blood or confirmed RAS mutation with or without elevated alpha/beta DNTs.
- Be enrolled in ALPS natural history protocol #93-I-0063.
- Have 1 or more of the following:
- Sudden enlargement of at least 1 lymph node or group of lymph nodes over baseline.
- Systemic symptoms suspicious for lymphoma (i.e., loss of weight, loss of appetite, fatigue, night sweats, fever, and pruritus).
- A histologically proven diagnosis of lymphoma or other malignancy.
- Be 5 years of age or older.
Who cannot join
- Patients will be excluded if any of the following is present:
- Concurrent proven 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.
- Hyperglycemia (regardless of etiology) determined by fasting glucose of >200 mg/dL
- Weight in excess of 400 lb, which will exceed the weight limit for the scanner table.
- Pregnancy or breast-feeding. For women of childbearing potential, a negative urine or serum pregnancy test is required within 24 hours prior to an FDG-PET/CT scan.
About this study
Official title: Use of Fluorodeoxyglucose Positron Emission Tomography With Computed Tomography for the Evaluation of Autoimmune Lymphoproliferative Syndrome Lymphadenopathy Suggestive of Lymphoma
Background:
- Autoimmune lymphoproliferative syndrome (ALPS) is a genetic disorder of the lymph system. People with ALPS often have swollen lymph nodes, especially in the neck and armpit. They also have a much higher risk of developing lymphoma. It is not always easy to determine whether the swollen lymph nodes are caused by ALPS or by lymphoma. Researchers want to see whether different imaging studies can show the difference between ALPS and lymphoma. The studies used will be positron emission tomography (PET) and computed tomography (CT). Researchers will use a drug called fluorodeoxyglucose (FDG) to look at the lymph nodes.
Read more
Objectives:
- To see how well imaging studies can distinguish between swollen lymph nodes caused by ALPS or by lymphoma.
Eligibility:
- Individuals must be 5 years of age or older and enrolled on the National Institutes of Health natural history study of ALPS.
- Participants should either have lymphoma or have symptoms that suggest possible lymphoma.
Design:
- Participants will be screened with a physical exam and medical history. Blood and urine samples will be collected.
- Participants will have an FDG-PET/CT scan. It will be performed according to standard procedures.
- If the results of the scan do not show lymphoma, participants will stay on the study for 1 year for clinical follow up. They may have a second FDG-PET/CT scan if there is a change in symptoms. Such changes include further enlargement of lymph nodes, unexplained fevers, or weight loss.
- If the results of the scan show evidence of new or worsening lymphoma, treatment on this study will end. Further tests based on clinical symptoms, including a lymph node biopsy, may be done under the ALPS natural history study to rule out or make a diagnosis of lymphoma.
Read the detailed description
The Autoimmune Lymphoproliferative Syndrome (ALPS) is an inherited disorder associated with defective lymphocyte apoptosis, which is clinically characterized by prominent nonmalignant 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 lymphoma (NHL) and Hodgkin lymphoma (HL). 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. Fluorodeoxyglucose positron emission tomography combined with computed tomography (FDG-PET/CT) is a noninvasive test that may help us discriminate benign from malignant lymphadenopathy in patients with ALPS.
For patients with ALPS and clinical symptoms suggestive of lymphoma, such as a sudden increase in focal lymphadenopathy and/or systemic B symptoms associated with lymphoma, we want to investigate whether FDG-PET/CT is useful in determining a plan of action by assisting in locating the most active lymph node and determining whether a surgical biopsy is warranted. Under this protocol, FDG-PET/CT scans will be done to rule out lymphoma. A lymph node biopsy may be necessary to determine the pathology of the lymph node and will not be done for research purposes alone.
- Conditions
- Autoimmune Lymphoproliferative Syndrome, Lymphoma
- Keywords
- PET/CT, Lymphoma, Lymphadenopathy, ALPS
- Medical subject headings
- Autoimmune Lymphoproliferative Syndrome, Lymphoma, Lymphadenopathy
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted27 Aug 2012 (estimated)
- Study start6 Aug 2012
- Study completion27 Feb 2015
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 Autoimmune Lymphoproliferative SyndromeStudies 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
- Time perspective
- Prospective
- Enrolment
- 0 participants (actual)
Sponsor and investigators
- Sponsor type
- US National Institutes of Health
- Responsible party
- Sponsor
- Investigators
- V. Koneti Rao, M.D., Principal investigator, National Institute of Allergy and Infectious Diseases (NIAID)
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT01672918
- Sponsor's protocol ID
- 120184
- Other IDs
- 12-I-0184
- Status last verified
- 27 Feb 2015
- First submitted
- 23 Aug 2012
- Last updated
- 5 Dec 2019
- 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 Dec 2019. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Use of Fluorodeoxyglucose Positron Emission Tomography With Computed Tomography for the Evaluation of Autoimmune Lymphoproliferative Syndrome Lymphadenopathy Suggestive of Lymphoma
- ClinicalTrials.gov ID
- NCT01672918
- Acronym / sponsor ID
- 120184
- Conditions
- Autoimmune Lymphoproliferative Syndrome, Lymphoma
- Keywords
- PET/CT, Lymphoma, Lymphadenopathy, ALPS
- Study type
- Observational
- Recruitment status
- Withdrawn
- Enrolment
- 0 participants (actual)
- Lead sponsor
- National Institute of Allergy and Infectious Diseases (NIAID)US National Institutes of Health
- Study start
- 6 Aug 2012
- Study completion
- 27 Feb 2015
- First posted
- 27 Aug 2012 (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.
Background:
- Autoimmune lymphoproliferative syndrome (ALPS) is a genetic disorder of the lymph system. People with ALPS often have swollen lymph nodes, especially in the neck and armpit. They also have a much higher risk of developing lymphoma. It is not always easy to determine whether the swollen lymph nodes are caused by ALPS or by lymphoma. Researchers want to see whether different imaging studies can show the difference between ALPS and lymphoma. The studies used will be positron emission tomography (PET) and computed tomography (CT). Researchers will use a drug called fluorodeoxyglucose (FDG) to look at the lymph nodes.
Objectives:
- To see how well imaging studies can distinguish between swollen lymph nodes caused by ALPS or by lymphoma.
Eligibility:
- Individuals must be 5 years of age or older and enrolled on the National Institutes of Health natural history study of ALPS.
- Participants should either have lymphoma or have symptoms that suggest possible lymphoma.
Design:
- Participants will be screened with a physical exam and medical history. Blood and urine samples will be collected.
- Participants will have an FDG-PET/CT scan. It will be performed according to standard procedures.
- If the results of the scan do not show lymphoma, participants will stay on the study for 1 year for clinical follow up. They may have a second FDG-PET/CT scan if there is a change in symptoms. Such changes include further enlargement of lymph nodes, unexplained fevers, or weight loss.
- If the results of the scan show evidence of new or worsening lymphoma, treatment on this study will end. Further tests based on clinical symptoms, including a lymph node biopsy, may be done under the ALPS natural history study to rule out or make a diagnosis of lymphoma.
Detailed description
The Autoimmune Lymphoproliferative Syndrome (ALPS) is an inherited disorder associated with defective lymphocyte apoptosis, which is clinically characterized by prominent nonmalignant 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 lymphoma (NHL) and Hodgkin lymphoma (HL). 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. Fluorodeoxyglucose positron emission tomography combined with computed tomography (FDG-PET/CT) is a noninvasive test that may help us discriminate benign from malignant lymphadenopathy in patients with ALPS.
For patients with ALPS and clinical symptoms suggestive of lymphoma, such as a sudden increase in focal lymphadenopathy and/or systemic B symptoms associated with lymphoma, we want to investigate whether FDG-PET/CT is useful in determining a plan of action by assisting in locating the most active lymph node and determining whether a surgical biopsy is warranted. Under this protocol, FDG-PET/CT scans will be done to rule out lymphoma. A lymph node biopsy may be necessary to determine the pathology of the lymph node and will not be done for research purposes alone.
Study design
- Time perspective
- Prospective
Who may qualify
- Ages
- 5 to 99 years
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Child, Adult, Older adult
Key inclusion criteria (8 listed)
- Fulfill current criteria for the diagnosis of ALPS, which includes documented chronic nonmalignant lymphadenopathy and/or splenomegaly, and either greater than or equal to 1.5% T-cell receptor alpha/beta+ DNTs in the peripheral blood or confirmed RAS mutation with or without elevated alpha/beta DNTs.
- Be enrolled in ALPS natural history protocol #93-I-0063.
- Have 1 or more of the following:
- Be 5 years of age or older.
Key exclusion criteria (5 listed)
- Concurrent proven 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.
- Hyperglycemia (regardless of etiology) determined by fasting glucose of >200 mg/dL
- Weight in excess of 400 lb, which will exceed the weight limit for the scanner table.
- Pregnancy or breast-feeding. For women of childbearing potential, a negative urine or serum pregnancy test is required within 24 hours prior to an FDG-PET/CT scan.
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 was withdrawn before enrolling participants, so there are no results.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 5 Dec 2019.
- 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)
Questions about this trial
What is this clinical trial studying?
An observational study of Autoimmune Lymphoproliferative Syndrome and Lymphoma, run by National Institute of Allergy and Infectious Diseases (NIAID).
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 8 inclusion and 5 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: withdrawn, as of the sponsor's last update on 5 Dec 2019. Status can change between updates, so confirm with the study contact before making plans.
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 was withdrawn before enrolling participants, so there are no results.
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 NCT01672918, 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 Dec 2019; AzTrials last refreshed its copy on 26 Oct 2026.