What is the role of CT and PET scanning in the workup of autoimmune lymphoproliferative syndrome (ALPS)?

Updated: Jul 29, 2019
  • Author: Akaluck Thatayatikom, MD, RhMSUS; Chief Editor: Harumi Jyonouchi, MD  more...
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Although imaging studies are not used in establishing a diagnosis of ALPS, once the diagnosis has been made, it is important to obtain baseline and periodic computed tomography (CT) scans. CT of the neck, chest, abdomen and pelvis at the time of diagnosis help establish the extent and location of the patient’s lymphadenopathy.

Because of the increased risk of lymphoma in patients with ALPS, frequent surveillance with serial CT scans is an important part of the long-term management of these patients. As a consequence of the chronic fluctuating nature of their lymphadenopathy, ALPS patients may undergo repeated lymph node biopsies to help rule out lymphoma if they develop systemic symptoms in addition to focal changes of adenopathy.

Positron emission tomography (PET) using18 fluoro-2-deoxy-D-glucose (FDG), or FDG-PET, is the standard for staging and follow-up evaluations of cancers, including lymphomas. FDG-PET can be used as a screening tool for malignancy in ALPS to minimize LN biopsies; however, it can not accurately distinguish benign from malignant lymphoproliferation as ALPS lymphadenopathy is PET-avid. [6]

Positron emission tomography (PET) superimposed ov Positron emission tomography (PET) superimposed over a CT scan from a patient with autoimmune lymphoproliferative syndrome (ALPS). Note the massive cervical adenopathy. PET scans may be used as a screening tool in patients with autoimmune lymphoproliferative syndrome to decrease the number of lymph node biopsies used in screening for malignancy.

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