Which histologic findings are characteristic of autoimmune lymphoproliferative syndrome (ALPS)?

Updated: Jul 29, 2019
  • Author: Akaluck Thatayatikom, MD, RhMSUS; Chief Editor: Harumi Jyonouchi, MD  more...
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The elevated percentage of TCR α/β DNT cells caused by the lymphocyte dysregulation seen in ALPS results in fairly characteristic histopathologic findings on lymph node biopsy. These findings include follicular hyperplasia and paracortical expansion with a mixed infiltrate containing the DNT cells. This specific histologic pattern can help distinguish ALPS from other benign and malignant lymphoproliferative lesions.

Evaluation of lymph node tissue for clonality by means of immunoglobulin and TCR gene rearrangements and cytogenetic analysis for chromosomal aneuploidy are also important for helping to rule out the diagnosis of lymphoma in ALPS patients who have chronic lymphadenopathy. [41]

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