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. 2020 Oct 11;12(10):2921.
doi: 10.3390/cancers12102921.

Epidemiology of Cutaneous T-Cell Lymphomas: A Systematic Review and Meta-Analysis of 16,953 Patients

Gabor Dobos  1   2   3 , Anne Pohrt  4 , Caroline Ram-Wolff  1 , Céleste Lebbé  1   2   3 , Jean-David Bouaziz  1   2   3 , Maxime Battistella  2   3   5 , Martine Bagot  1   2   3 , Adèle de Masson  1   2   3
Affiliations

Epidemiology of Cutaneous T-Cell Lymphomas: A Systematic Review and Meta-Analysis of 16,953 Patients

Gabor Dobos et al. Cancers (Basel). .

Abstract

Cutaneous T-cell lymphomas (CTCL) are a heterogenous group of rare diseases. Many studies have reported on local epidemiology or geographic clustering, however we lack information from a global perspective. A systematic review and meta-analysis was conducted in Medline and the Cochrane Library based on a previously registered protocol and according to the preferred reporting of items for systematic reviews and meta-analyses (PRISMA). We selected publications that enrolled at least 100 patients with primary cutaneous lymphomas according to the current classifications. The relative frequencies (proportions) of subtypes were compared between studies and geographic regions in a meta-analysis. In total, 26 studies met our inclusion criteria, reporting on altogether 16,953 patients. Within primary cutaneous lymphomas, CTCL appeared to be 15% more frequent in Asian populations. Mycosis fungoides (MF) accounted for 62% of CTCL, with an important heterogeneity in frequencies between studies and continents. The proportion of Sézary syndrome (SS) was 3%, stable worldwide. Rare CTCL, such as NK/T-cell lymphoma or subcutaneous panniculitis-like lymphoma, were more frequent in Asian studies. This global meta-analysis of CTCL confirmed the predominance of CTCL among primary cutaneous lymphomas (83% on average) in the three analyzed continents, most of which were MF cases. It revealed the same proportions of SS across continents, and the heterogeneity of MF frequencies, suggesting the possible role of environmental factors in the pathophysiology of the latter. Registration number: CRD42020148295 (PROSPERO).

Keywords: cutaneous T-cell lymphomas; lymphomas; mycosis fungoides; skin; systematic review.

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Conflict of interest statement

The authors declare no conflict of interest. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript, or in the decision to publish the results.

Figures

Figure 1
Figure 1
Flowchart of study selection according to the preferred reporting of items for systematic reviews and meta-analyses (PRISMA).
Figure 2
Figure 2
Meta-analysis of the proportion of CTCL compared to all primary cutaneous lymphomas and overview of the subgroups.
Figure 3
Figure 3
Meta-analyses of the proportion of mycosis fungoides (MF) and Sézary syndrome (SS) compared to CTCL.
Figure 4
Figure 4
Meta-analyses of the proportions of CD30+ lymphoproliferative disorder subtypes, primary cutaneous anaplastic large cell lymphoma (ALCL) and lymphomatoid papulosis (LyP) compared to CTCL.
Figure 5
Figure 5
Meta-analyses of the proportion of rare CTCL, subcutaneous panniculitis-like T-cell lymphoma (SPCTL) and NK/T-cell lymphoma compared to CTCL.
See this image and copyright information in PMC

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