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Use of Fluorodeoxyglucose Positron Emission Tomography With Computed Tomography for the Evaluation of Autoimmune Lymphoproliferative Syndrome Lymphadenopathy Suggestive of Lymphoma.

5 Years
Open (Enrolling)
Autoimmune Lymphoproliferative Syndrome, Lymphoma

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Trial Information

Use of Fluorodeoxyglucose Positron Emission Tomography With Computed Tomography for the Evaluation of Autoimmune Lymphoproliferative Syndrome Lymphadenopathy Suggestive of Lymphoma.

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

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.

Inclusion Criteria


To qualify for enrollment, patients must meet all of the following:

1. 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.

2. Be enrolled in ALPS natural history protocol #93-I-0063.

3. Have 1 or more of the following:

1. Sudden enlargement of at least 1 lymph node or group of lymph nodes over

2. Systemic symptoms suspicious for lymphoma (i.e., loss of weight, loss of
appetite, fatigue, night sweats, fever, and pruritus).

3. A histologically proven diagnosis of lymphoma or other malignancy.

4. Be 5 years of age or older.


Patients will be excluded if any of the following is present:

1. 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.

2. Hyperglycemia (regardless of etiology) determined by fasting glucose of > 200 mg/dL

3. Weight in excess of 400 lb, which will exceed the weight limit for the scanner table.

4. 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.

Type of Study:


Study Design:

Time Perspective: Prospective

Principal Investigator

V. Koneti Rao, M.D.

Investigator Role:

Principal Investigator

Investigator Affiliation:

National Institute of Allergy and Infectious Diseases (NIAID)


United States: Federal Government

Study ID:




Start Date:

August 2012

Completion Date:

Related Keywords:

  • Autoimmune Lymphoproliferative Syndrome
  • Lymphoma
  • PET/CT
  • Lymphoma
  • Lymphadenopathy
  • ALPS
  • Lymphoma
  • Autoimmune Lymphoproliferative Syndrome



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