Clinical Characteristics and Prognosis of Patients With Primary Cutaneous T-cell Lymphoma

Objective To investigate the clinical features, treatment, and prognosis of primary cutaneous T-cell lymphoma （CTCL）. Methods Totally 15 patients with primary CTCL diagnosed by skin biopsy in the affiliated Hospital of Xuzhou Medical University from November 2009 to May 2019 were retrospectively analyzed to describe this rare tumor. Results The main results were as follows: 8 were males and 7 were females, and the median age was 50 years old. The vast majority of patients （13/15） see a doctor for skin lesions. The skin lesions of different subtypes of CTCL vary in shape. The main manifestation of mycosis fungoides （MF） was plaque （83.3%）; Subcutaneous panniculitis-like T-cell lymphoma （SPTCL） was mainly characterized by multiple subcutaneous nodules of the extremities （75%）; peripheral T-cell lymphoma not otherwise specified （PTCL-NOS） lesions were polymorphic. The main lesions were limb （n=7） and whole body （n=3）. The clinicopathological features of different subtypes of CTCL were different. T cell antigen was mainly expressed on the surface of tumor cells. The positive index of Ki-67 was 5%~80%. Epstein-Barr encoding region （EBER） was positive in only 1 case. The median follow-up time was 54.3 months, 2-year overall survival （OS） was 66.7%, and 2-year progression-free survival （PFS） was 43.9%. The median survival time of 7 dead patients was 18 months （range 14.0~57.2）. The 4-year OS （P=0.045 4） and PFS （P=0.002 3） of patients in the indolent or low-risk group were significantly better than those in the aggressive or high-risk group, and there were significant differences in PFS among MF, SPTCL, and PTCL-NOS （P=0.033 4）. Conclusions In this study, except for MF or SPTCL, the other CTCL subtype had strong invasiveness and poor prognosis. The manifestations and histopathological morphology of various subtypes of skin lesions were diversified. Clinical manifestations, histopathological examination, and immunophenotype should be combined in the diagnosis to avoid missed diagnosis and misdiagnosis.