Neoplastic Cells of Primary Cutaneous CD4+ Small/Medium-sized Pleomorphic T-cell Lymphoma Lack Expression of Follicular T-helper Cell Defining Chemokine Receptor CXCR5

Primary cutaneous CD4+ small/medium pleomorphic T‐cell lymphoproliferative disorder: Where do we stand? A systematic review

“…reported that such TFH cells specifically lack expression of the TFH‐cell‐defining chemokine receptor (CXCR5), which is a very characteristic finding for PCSMP‐TLPD. This lymphoproliferative disorder is always negative for Epstein‐Barr virus . Calcineurin/nuclear factor of activated T‐cells‐c1 (NFATc1) was recently shown to produce significant nuclear staining of lymphocytes (nuclear in neoplastic cells; cytoplasmic in both neoplastic and reactive cells) in PCSMP‐TLPD, which would be helpful in differentiating it from MF (predominantly cytoplasmic in both neoplastic and reactive cells) and pseudolymphoma (almost exclusively cytoplasmic) .…”

“…Furthermore, Krenács et al. used microdissection to show that T‐cell clonality is linked to PD1+ cells, suggesting that they are the cells of origin in PCSMP‐TLPD . Additionally, the clonality of Ig heavy and/or light chains has been tested in some patients, and was found to be mostly polyclonal .…”

“…The optimal treatment for PCSMP‐TLPD has not yet been defined. However, various therapeutic options have been used for solitary lesions, including surgical excision, radiotherapy or a combination of both , as well as topical, intralesional or oral steroids , doxycycline monohydrate , amoxicillin , cryocautery and bexarotene . Systemic chemotherapy (including cyclophosphamide either as monotherapy or in association with prednisolone) , multi‐agent chemotherapy (as cyclophosphamide, hydroxydaunomycin, oncovin and prednisone (CHOP) therapy) and systemic interferon‐alfa have been used for patients with multiple aggressive lesions, which are now considered mostly as true lymphomas rather than cases of PCSMP‐TLPD.…”

Primär kutane CD4+ klein‐ bis mittelgroßzellige pleomorphe T‐Zell‐Lymphoproliferation: Wo stehen wir? Eine systematische Übersicht

“…Allerdings berichteten Krenács et al. , dass speziell bei diesen TFH‐Zellen der die TFH‐Zellen kennzeichnende Chemokinrezeptor (CXCR5) fehlt. Dies ist bei PCSMP‐TLPD ein sehr charakteristischer Befund.…”

“…Darüber hinaus zeigten Krenács et al. mithilfe der Mikrodissektion, dass die T‐Zell‐Klonalität an PD1+‐Zellen gebunden ist, was darauf hinweist, dass diese die Ursprungszellen bei PCSMP‐TLPD sein könnten. Darüber hinaus waren die bei manchen Patienten untersuchten schweren und/oder leichten Ig‐Ketten vorwiegend polyklonal .…”