Background: Primary cutaneous T-cell lymphoma (CTCL) is a group of disorder characterized by proliferation of malignant T lymphocytes primarily in the skin. The frequency and clinicopathological characteristics of CTCL vary in different countries. Aim: The objective of this study was to determine the clinical pattern of CTCL in the Department of Dermatology, Hospital Kuala Lumpur, Malaysia. Methods: A retrospective review of all patients diagnosed with CTCL between January 2005 and December 2017 was conducted in the Department of Dermatology, Hospital Kuala Lumpur. The diagnosis was confirmed histologically and classified based on WHO-EORTC classification. Results: A total of 102 patients were identified. Mean age at presentation was 43.2 ± 20.8 years. 62.7% of patients were male and 37.3% female (male:female ratio 1.7:1.0). 50.0% of patients were Malay, 28.4% Chinese, 15.7% Indian and 5.9% from other ethnic groups. The commonest type of CTCL was mycosis fungoides (MF), which accounted for 82.4% of patients. This was followed by primary cutaneous CD30+ lymphoproliferative disorder (primary cutaneous anaplastic large cell lymphoma in 7.8%, and lymphomatoid papulosis in 2.9% of patients). Subcutaneous panniculitis-like T-cell lymphoma was seen in 3.9% of patients and Sézary syndrome seen in 2.9% of patients. 36.9% of patients with MF had hypopigmented MF variant. Other variants of MF were not common (erythrodermic MF in 6.0%, poikilodermatous MF in 6.0%, granulomatous MF in 3.6% and pagetoid reticulosis in 1.2% of patients). Majority of patients (68.7%) were in early stages of disease (Stage IA-IIA). Most patients had skin directed therapy, which included topical steroids in 79.2%, NBUVB in 39.6% and PUVA in 31.7% of patients. Commonest systemic agent used was methotrexate (10.8%), followed by acitretin and interferon (5.0% each). Only 2.0% of patients had localized radiotherapy and 1.0% of patients had total skin electron beam therapy. Chemotherapy was given in 6.9% of patients. Data on outcome was available for 81 patients. 19.8% of patients had complete remission at last follow-up, 48.1% had partial remission, 17.3% had refractory or progressive disease and 14.8% died of the disease. 19.8% of patients were lost to follow-up. Conclusion: Patients with CTCL from our institution were younger, with a mean age at presentation of 43.2 ± 20.8 years. This is in contrast with data from Surveillance, Epidemiology and End Results (SEER) registries between 1973 and 2009, which indicated that CTCL tends to occur in an older age group, above 55 years of age. Similar to other studies, we also found CTCL to be commoner in male patients (male:female ratio 1.7:1.0). The commonest type of CTCL in our patients was mycosis fungoides (MF). 36.9% of patients with MF had hypopigmented MF variant, which is consistent with other studies where hypopigmented MF were found almost exclusively in persons of color.
Increased Expression of CTLA-4 in Malignant T Cells from Patients with Mycosis Fungoides – Cutaneous T-Cell Lymphoma
