BACKGROUND: Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (CD4+ PCSM-LPD) is defined by a predominance of small- to medium-sized CD4+ pleomorphic T cells and a favorable clinical course. OBJECTIVE: We performed a retrospective analysis of 6 patients with CD4+ PCSM-LPD and reviewed the literature to address questions about its diagnosis, treatment, and prognosis. METHODS: Patients were 3 men and 3 women with a median age of 50 years. All patients presented with a single erythematous nodule, localised on the head in 4 patients and the upper trunk in 2 cases. No patients showed extracutaneous disease at any evaluation. Histopathologic features were characterised by nodular, diffuse, or, in 1 case, a superficial dense infiltrate of small/medium-sized pleomorphic CD4+/PD1+ T lymphocytes. T-cell receptor clonality was demonstrated in 5 cases. Treatment was surgical excision in 5 cases and radiotherapy in 1 case. RESULTS: All patients achieved complete resolution without relapses, during a median follow-up of 3 years. A review of the literature confirmed that CD4+ PCSM-LPD presents predominantly with a solitary nodular lesion on the face, neck, or upper trunk in adult patients. Surgical excision is the preferred treatment. Spontaneous resolution after biopsy may occur. CONCLUSIONS: CD4+ PCSM-LPD is a rare disorder with a favorable course.

Primary Cutaneous CD4+ Small/Medium Pleomorphic T-Cell Lymphoproliferative Disorder: A Case Series

Maurelli, M;Colato, C;Gisondi, P;Girolomoni, G.
2017-01-01

Abstract

BACKGROUND: Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (CD4+ PCSM-LPD) is defined by a predominance of small- to medium-sized CD4+ pleomorphic T cells and a favorable clinical course. OBJECTIVE: We performed a retrospective analysis of 6 patients with CD4+ PCSM-LPD and reviewed the literature to address questions about its diagnosis, treatment, and prognosis. METHODS: Patients were 3 men and 3 women with a median age of 50 years. All patients presented with a single erythematous nodule, localised on the head in 4 patients and the upper trunk in 2 cases. No patients showed extracutaneous disease at any evaluation. Histopathologic features were characterised by nodular, diffuse, or, in 1 case, a superficial dense infiltrate of small/medium-sized pleomorphic CD4+/PD1+ T lymphocytes. T-cell receptor clonality was demonstrated in 5 cases. Treatment was surgical excision in 5 cases and radiotherapy in 1 case. RESULTS: All patients achieved complete resolution without relapses, during a median follow-up of 3 years. A review of the literature confirmed that CD4+ PCSM-LPD presents predominantly with a solitary nodular lesion on the face, neck, or upper trunk in adult patients. Surgical excision is the preferred treatment. Spontaneous resolution after biopsy may occur. CONCLUSIONS: CD4+ PCSM-LPD is a rare disorder with a favorable course.
diagnosis; lymphoma; lymphoproliferative disease; prognosis; therapy
File in questo prodotto:
File Dimensione Formato  
Primary Cutaneous CD4+ SmallMedium Pleomorphic T-Cell Lymphoproliferative Disorder J Cutan Med Surg 2017.pdf

non disponibili

Tipologia: Documento in Post-print
Licenza: Accesso ristretto
Dimensione 2.58 MB
Formato Adobe PDF
2.58 MB Adobe PDF   Visualizza/Apri   Richiedi una copia

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11562/969775
Citazioni
  • ???jsp.display-item.citation.pmc??? ND
  • Scopus 20
  • ???jsp.display-item.citation.isi??? ND
social impact