What is the role of lymph node biopsy 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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Answer

Lymph node biopsies are typically performed in ALPS patients. Such biopsies reveal findings unique to ALPS (see Histologic Findings). If the biopsy findings obtained in a patient with lymphadenopathy are consistent with a diagnosis of ALPS, further laboratory testing, including flow cytometric immunophenotyping and genetic testing, is warranted.

At the time of diagnosis, biopsy is used to look for histology suggestive of ALPS, as well as to rule out lymphoma. ALPS is associated with both chronic lymphadenopathy and a significant increase in the risk for lymphoma. In view of these 2 associations, lymph node biopsy may be required if a sudden or dramatic change in a node is noted, particularly if these changes are associated with concerning constitutional symptoms, such as weight loss, fever, and night sweats. [44]

Serial CT and PET may be used as tools to mitigate the tendency to perform repeated lymph node biopsies while still effectively monitoring these patients for malignancy.


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