Stanislav N Tolkachjov1, Roger H Weenig2, Nneka I Comfere3. 1. Department of Dermatology, Mayo Clinic, Rochester, Minnesota. 2. Associated Skin Care Specialists PA, Fridley, Minnesota. 3. Department of Dermatology, Mayo Clinic, Rochester, Minnesota; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota. Electronic address: comfere.nneka@mayo.edu.
Abstract
BACKGROUND: Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) is a rare and aggressive disease either originating in or secondarily involving the skin. OBJECTIVE: We sought to assess clinical, histopathologic, and prognostic features of patients with cutaneous PTCL-NOS. METHODS: This was a retrospective chart review of patients with cutaneous PTCL-NOS between 1993 and 2013. RESULTS: Thirty patients with PTCL-NOS were included. Fourteen had skin-only disease and 15 had concurrent skin and systemic disease at presentation. In primary cutaneous PTCL-NOS, the overall survival rate at 5 years was 61% (95% confidence interval, 37-100%; number still at risk, 2). The median overall survival was 5.6 years. Patients were diagnosed a median of 2.4 months from symptom onset. Patients with concurrent disease died a median of 2.1 years after diagnosis. The estimated overall survival rate at 5 years after diagnosis was 29% (95% confidence interval, 13-67%; number at risk, 3). The median overall survival was 3.9 years. Patients were diagnosed a median of 6 months from symptom onset, with a 53% increased risk of death for each year from symptom onset to diagnosis. LIMITATIONS: This was a retrospective study with a limited number of cases. CONCLUSIONS: Age at diagnosis and B-symptoms predict poor survival in patients with cutaneous PTCL-NOS. In addition, poorer survival is observed in patients with multifocal lesions and concomitant skin and systemic PTCL-NOS.
BACKGROUND: Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) is a rare and aggressive disease either originating in or secondarily involving the skin. OBJECTIVE: We sought to assess clinical, histopathologic, and prognostic features of patients with cutaneous PTCL-NOS. METHODS: This was a retrospective chart review of patients with cutaneous PTCL-NOS between 1993 and 2013. RESULTS: Thirty patients with PTCL-NOS were included. Fourteen had skin-only disease and 15 had concurrent skin and systemic disease at presentation. In primary cutaneous PTCL-NOS, the overall survival rate at 5 years was 61% (95% confidence interval, 37-100%; number still at risk, 2). The median overall survival was 5.6 years. Patients were diagnosed a median of 2.4 months from symptom onset. Patients with concurrent disease died a median of 2.1 years after diagnosis. The estimated overall survival rate at 5 years after diagnosis was 29% (95% confidence interval, 13-67%; number at risk, 3). The median overall survival was 3.9 years. Patients were diagnosed a median of 6 months from symptom onset, with a 53% increased risk of death for each year from symptom onset to diagnosis. LIMITATIONS: This was a retrospective study with a limited number of cases. CONCLUSIONS: Age at diagnosis and B-symptoms predict poor survival in patients with cutaneous PTCL-NOS. In addition, poorer survival is observed in patients with multifocal lesions and concomitant skin and systemic PTCL-NOS.
Authors: Ji-Hye Park; YoungHwan Choi; Hyun Jeong Byun; Se Jin Oh; Jong Hee Lee; Dong-Youn Lee Journal: Ann Dermatol Date: 2020-06-30 Impact factor: 1.444