18F-FDG PET Imaging Features of Patients With Autoimmune Lymphoproliferative Syndrome.

ABSTRACT:

Introduction

Autoimmune lymphoproliferative syndrome (ALPS) is a rare immune dysregulatory condition, usually presenting in childhood with massive lymphadenopathy, splenomegaly, and an increased incidence of lymphoma. Methods to differentiate between benign ALPS adenopathy and lymphoma are needed. To this end, we evaluated the usefulness of FDG PET.

Methods

We prospectively evaluated 76 ALPS/ALPS-like patients including FS-7-associated surface antigen (FAS) germline mutation with (n = 4) and without lymphoma (n = 50), FAS-somatic (n = 6), ALPS-unknown (n = 6), and others (n = 10) who underwent FDG PET. Uptakes in 14 nodal sites, liver, and spleen were determined.

Results

In 76 ALPS patients, FDG PET showed uptake in multiple nodal sites in all but 1 patient. The highe

SUBMITTER: Carrasquillo JA 

PROVIDER: S-EPMC8189160 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature