Know Cancer

or
forgot password

Study of Fluorodeoxyglucose Positron Emission Tomography (FDG-PET) for the Evaluation of the Autoimmune Lymphoproliferative Syndrome (ALPS) and ALPS-Associated Lymphoma


N/A
8 Years
N/A
Not Enrolling
Both
Lymphoproliferative Disorders, Lymphoma

Thank you

Trial Information

Study of Fluorodeoxyglucose Positron Emission Tomography (FDG-PET) for the Evaluation of the Autoimmune Lymphoproliferative Syndrome (ALPS) and ALPS-Associated Lymphoma


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.

Inclusion Criteria


- 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.

Type of Study:

Observational

Study Design:

N/A

Principal Investigator

Amy D Klion, M.D.

Investigator Role:

Principal Investigator

Investigator Affiliation:

National Institute of Allergy and Infectious Diseases (NIAID)

Authority:

United States: Federal Government

Study ID:

020308

NCT ID:

NCT00068146

Start Date:

September 2002

Completion Date:

November 2012

Related Keywords:

  • Lymphoproliferative Disorders
  • Lymphoma
  • Adenopathy
  • Genetic Disorder
  • Apoptosis
  • Diagnostic Imaging
  • Hodgkins
  • Autoimmune Lymphoproliferative
  • Syndrome
  • ALPS
  • ALPS-Associated Lymphoma
  • Lymphoma
  • Lymphoproliferative Disorders
  • Autoimmune Lymphoproliferative Syndrome

Name

Location

National Institutes of Health Clinical Center, 9000 Rockville PikeBethesda, Maryland  20892