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Updated: Jun 28, 2026

Assessing the Development of Murine Plasmacytoid Dendritic Cells in Peyer's Patches Using Adoptive Transfer of Hematopoietic Progenitors
Published on: March 17, 2014
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.
Objective:
Describe the presence of plasmacytoid dendritic cells and type 1 interferon and the activation of interferon signaling pathway in lymphatic malformations.
Design:
Retrospective case series.
Materials:
Nineteen lymphatic malformations with matching clinical data were compared to control tissue (normal skin and lymph node).
Methods:
Paraffin-embedded tissue was evaluated for plasmacytoid dendritic cells, type 1 interferon, and type 1 interferon signaling with immunohistochemistry and RNA in situ hybridization. Staining results were compared with controls and correlated with clinical data with descriptive statistics and Mann-Whitney tests.
Results:
Lymphatic malformations had increased staining for plasmacytoid dendritic cells, interferons alpha and beta, and phosphorylated signal transducer and activator of transcription 1 compared with controls (P < 0.05). Significantly increased interferon beta staining was present in recurrent lymphatic malformation tissue compared with nonrecurrent lymphatic malformations.
Conclusion:
Lymphatic malformations have unusual ongoing local immune activation, possibly affecting recurrence.
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