Abstract
Background Primary cutaneous lymphomas (PCLs) refer to cutaneous lymphomas that primarily develop in the skin with no evidence of extracutaneous disease at the time of diagnosis. The epidemiological and clinical data of PCLs in Thailand are lacking. Objectives To evaluate the frequency, demographic data, and clinical characteristics of different subtypes of PCLs in a tertiary care university hospital. Methods In total, 137 patients with PCLs diagnosed in our hospital in 2008–2017 were retrospectively reviewed. Results Of the 137 patients, 57 (41.6%) were male and 80 (58.4%) were female (M : F = 1 : 1.4). The median age at diagnosis was 40 years. Most patients (134, 97.8%) had cutaneous T-cell lymphomas (CTCLs). Three patients (2.2%) had cutaneous B-cell lymphomas (CBCLs). The most common subtype was mycosis fungoides (MF) (67.9%), followed by subcutaneous panniculitis-like T-cell lymphoma (SPTCL) (21.2%), primary cutaneous anaplastic large cell lymphoma (pcALCL) (3.6%), lymphomatoid papulosis (LyP) (1.5%), primary cutaneous gamma/delta T-cell lymphoma (pcGDTCL) (1.5%), Sézary syndrome (SS) (0.7%), extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT) (0.7%), primary cutaneous peripheral T-cell lymphoma, not otherwise specified (pcPTCL-NOS) (0.7%), primary cutaneous diffuse large B-cell lymphoma, leg type (pcDLBCL-LT) (1.5%), and primary cutaneous follicle center lymphoma (pcFCL) (0.7%). Most patients with MF presented with early-stage disease (84.0%), with hypopigmented MF the most common variant (42.6%). Conclusions Compared to earlier Caucasian and Asian studies, the present study revealed a higher proportion of CTCL patients with a younger age at onset and a female predominance. MF was the most common CTCL subtype, followed by SPTCL. More than 80% of MF patients were diagnosed at an early stage.
Highlights
Primary cutaneous lymphomas (PCLs) are rare disorders, which refer to a group of extranodal non-Hodgkin lymphomas (NHLs) that primarily occur in the skin without evidence of extracutaneous disease at the time of diagnosis [1]
We identified a total of 137 patients diagnosed with PCLs from 2008 to 2017
cutaneous T-cell lymphomas (CTCLs) were the most common group of PCLs, comprising 134 (97.8%) patients, whereas only 3 (2.2%) patients were diagnosed with cutaneous B-cell lymphomas (CBCLs)
Summary
Primary cutaneous lymphomas (PCLs) are rare disorders, which refer to a group of extranodal non-Hodgkin lymphomas (NHLs) that primarily occur in the skin without evidence of extracutaneous disease at the time of diagnosis [1]. PCLs may resemble their nodal counterparts in clinical and immunohistopathological findings, but differ in terms of their clinical course, management, and prognosis. They tend to have a more indolent course and better prognosis for the most part. Primary cutaneous lymphomas (PCLs) refer to cutaneous lymphomas that primarily develop in the skin with no evidence of extracutaneous disease at the time of diagnosis. Most patients (134, 97.8%) had cutaneous T-cell lymphomas (CTCLs). Most patients with MF presented with early-stage disease (84.0%), with hypopigmented MF the most common variant (42.6%). More than 80% of MF patients were diagnosed at an early stage
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