Tertiary lymphoid structures are confined to patients presenting with unifocal Langerhans Cell Histiocytosis

Willemijn T. Quispel, Eline C. Steenwijk, Vincent van Unen, Susy J. Santos, Lianne Koens, Reina Mebius, R. Maarten Egeler, Astrid G.S. van Halteren

Onderzoeksoutput: Bijdrage aan tijdschriftArtikelpeer review


Langerhans cell histiocytosis (LCH) is a neoplastic myeloid disorder with a thus far poorly understood immune component. Tertiary lymphoid structures (TLS) are lymph node-like entities which create an immune-promoting microenvironment at tumor sites. We analyzed the presence and clinical relevance of TLS in n = 104 H&E-stained, therapy-naive LCH lesions of non-lymphoid origin and applied immunohistochemistry to a smaller series. Lymphoid-follicular aggregates were detected in 34/104 (33%) lesions. In line with the lymphocyte recruitment capacity of MECA-79+ high endothelial venules (HEVs), MECA-79+-expressing-LCH lesions (37/77, 48%) contained the most CD3+ T-lymphocytes (p = 0.003). TLS were identified in 8/15 lesions and contained T-and B-lymphocytes, Follicular Dendritic Cells (FDC), HEVs and the chemokines CXCL13 and CCL21 representing key cellular components and TLS-inducing factors in conventional lymph nodes (LN). Lymphoid-follicular aggregates were most frequently detected in patients presenting with unifocal LCH (24/70, 34%) as compared to patients with poly-ostotic or multi-system LCH (7/30, 23%, p = 0.03). In addition, patients with lymphoid-follicular aggregates-containing lesions had the lowest risk to develop new LCH lesions (p = 0.04). The identification of various stages of TLS formation within LCH lesions may indicate a key role for the immune system in controlling aberrant histiocytes which arise in peripheral tissues.

Originele taal-2Engels
Nummer van het tijdschrift8
StatusGepubliceerd - 2 aug. 2016
Extern gepubliceerdJa


Duik in de onderzoeksthema's van 'Tertiary lymphoid structures are confined to patients presenting with unifocal Langerhans Cell Histiocytosis'. Samen vormen ze een unieke vingerafdruk.

Citeer dit