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CD27/CD70 pathway activation in primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder
Julia Richter1, Ilske Oschlies1, Katharina Kock1
1Department of Pathology, Hematopathology Section, University Hospital Schleswig-Holstein, Kiel, Germany.
The Journal of Pathology
|November 7, 2023
Summary
Primary cutaneous CD4+ small or medium T-cell lymphoproliferative disorder (PCSM-LPD) shows distinct molecular features compared to aggressive Tfh lymphomas. CD70 pathway activation may drive the benign proliferation of PCSM-LPD.
Area of Science:
- Dermatology
- Oncology
- Immunology
Background:
- Primary cutaneous CD4+ small or medium T-cell lymphoproliferative disorder (PCSM-LPD) is a skin-confined clonal T-cell proliferation.
- PCSM-LPD exhibits T follicular helper (Tfh) cell markers but presents benignly, unlike aggressive Tfh lymphomas.
Purpose of the Study:
- To investigate molecular differences and similarities between PCSM-LPD and Tfh-derived lymphomas.
- To elucidate potential pathological mechanisms behind PCSM-LPD's benign clinical behavior.
Main Methods:
- Targeted next-generation sequencing of 19 genes in 38 PCSM-LPD samples.
- Gene expression profiling to identify genes in PD1-positive neoplastic cells.
- Multistain immunofluorescence to confirm gene expression in situ.
Main Results:
- PCSM-LPD rarely showed mutations common in other T-cell neoplasms.
- Invariable expression of LCK protein and co-expression of CD70/CD27 on PD1+ PCSM-LPD cells were observed.
- Evidence suggests autoactivation of the CD70 pathway.
Conclusions:
- PCSM-LPD possesses a unique mutation profile distinguishing it from disseminated Tfh lymphomas.
- CD70 signaling activation, also seen in cutaneous T-cell lymphoma, may drive PCSM-LPD proliferation.
- This suggests a potential therapeutic target for this benign neoplasm.
