How is autoimmune lymphoproliferative syndrome (ALPS) staged?

Updated: Jul 29, 2019
  • Author: Akaluck Thatayatikom, MD, RhMSUS; Chief Editor: Harumi Jyonouchi, MD  more...
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A specific staging system that can be used to establish a prognosis in patients with ALPS has not been established. However, the degree of generalized lymphadenopathy can be documented longitudinally in a consistent fashion by using the following guidelines:

  • Grade 1 - Few shotty nodes

  • Grade 2 - Multiple 1-2 cm nodes

  • Grade 3 - Multiple nodes, some larger than 2 cm

  • Grade 4 - Extensive visible adenopathy

This grading is best performed by using physical examination and CT scanning in the clinical setting. [45]

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