Fluorodeoxyglucose-Positron Emission Tomography (FDG-PET) to Evaluate Autoimmune Lymphoproliferative Syndrome (ALPS) and ALPS-associated Lymphoma
| Status: | Completed |
|---|---|
| Conditions: | Lymphoma, Hematology |
| Therapuetic Areas: | Hematology, Oncology |
| Healthy: | No |
| Age Range: | 8 - Any |
| Updated: | 10/8/2017 |
| Start Date: | September 19, 2002 |
| End Date: | November 26, 2012 |
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.
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.
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.
- INCLUSION CRITERIA:
ALPS Patients without Lymphoma:
Participants must:
1. Fulfill current criteria for the diagnosis of ALPS as follows:
1. Documented chronic nonmalignant lymphadenopathy and/or splenomegaly, and
2. Greater than or equal to 1.5 percent TCR alpha/beta+ CD4- CD8- T cells in the
peripheral blood.
2. Be enrolled in ALPS Natural History Protocol 93-I-0063.
3. 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.
4. 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.
5. 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:
1. Fulfill current criteria for the diagnosis of ALPS as follows:
1. Documented chronic nonmalignant lymphadenopathy and/or splenomegaly.
2. Greater than or equal to 1.5 percent TCR alpha/beta + CD4- CD8 -T cells in the
peripheral blood.
2. Be enrolled in ALPS Natural History Protocol 93-I-0063.
3. 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.
EXCLUSION CRITERIA:
1. Patient will be excluded if any of the following are present:
2. 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.
3. Active neoplasia other than lymphoma.
4. History of chemotherapy or radiation treated malignancy within 5 years prior to study
procedure, except for lymphoma.
5. 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.
6. Weights in excess of 136 kg, which will exceed the weight limit for the scanner table.
7. 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.
We found this trial at
1
site
9000 Rockville Pike
Bethesda, Maryland 20892
Bethesda, Maryland 20892
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