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.
Time Perspective: Prospective
V. Koneti Rao, M.D.
National Institute of Allergy and Infectious Diseases (NIAID)
United States: Federal Government
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