Plasmacytoid dendritic cells and interferon levels are increased in lymphatic malformations

Jo Nadine Fleming1, Sirkka Liisa Hostikka, Eunice Y Chen

  • 1Department of Pathology, University of Washington, Seattle, WA, USA.

Insights

Lymphatic malformations show increased immune cell activity and type 1 interferon signaling. This immune activation may be linked to lymphatic malformation recurrence.

Area of Science:

  • Immunology
  • Dermatology
  • Pathology

Background:

  • Lymphatic malformations (LMs) are congenital vascular anomalies.
  • The underlying pathophysiology of LMs is not fully understood.
  • Immune dysregulation may play a role in LM development and progression.

Purpose of the Study:

  • To investigate the presence and role of plasmacytoid dendritic cells (pDCs) and type 1 interferons (IFNs) in LMs.
  • To assess the activation of the IFN signaling pathway in LM tissue.
  • To correlate immune findings with clinical data, including recurrence.

Main Methods:

  • Retrospective case series of 19 LMs with clinical data.
  • Immunohistochemistry and RNA in situ hybridization on paraffin-embedded LM tissue.
  • Comparison with control tissues (normal skin and lymph node).

Main Results:

  • LMs exhibited increased pDCs, IFN-alpha, IFN-beta, and phosphorylated STAT1 compared to controls (P < 0.05).
  • Recurrent LMs showed significantly higher IFN-beta staining than non-recurrent LMs.
  • These findings indicate local immune activation within LMs.

Conclusions:

  • LMs demonstrate ongoing, localized immune activation.
  • Type 1 IFN signaling and pDCs are implicated in the pathogenesis of LMs.
  • This immune activation may contribute to LM recurrence.
Abstract