Frequency of primary cutaneous lymphoma variants in Austria: retrospective data from a dermatology referral centre between 2006 and 2013

Abstract

Background Primary cutaneous lymphomas (PCL) are a heterogenous group of rare lymphoid neoplasms with incomplete information on global and regional prevalence. The recently introduced lymphoma classifications define distinctive clinicopathological disease entities that should allow for more accurate epidemiological assessment. Objective The aim of this study was to evaluate the prevalence and clinical spectrum of PCL diagnosed and treated at the Department of Dermatology and Venereology in St. Pölten, Lower Austria, a dermatology referral centre providing secondary and tertiary care for a population of about 600 000. Methods In this retrospective study pathology reports, electronically archived between 2006 and 2013, were screened for the terms lymphoma, mycosis fungoides (MF) and lymphomatoid papulosis (LyP). Patients were diagnosed according to the current WHO‐EORTC classification for cutaneous lymphomas and results were compared with data from European, US and Asian centres. Results Among 86 patients with PCL (age 58.3 ± 17.35 years, mean ± SD; women 38%, n = 33; men 62%, n = 53) 83% (n = 71) were classified as cutaneous T‐cell lymphomas (CTCL) and 17% (n = 15) as cutaneous B‐cell lymphomas (CBCL). Nine patients with CTCL showed associated haematological disorders and malignomas. Among 47 MF patients following variants were observed: pilotropic MF (n = 2), follicular mucinosis (n = 1), unilesional MF (n = 1), large‐cell transformation (n = 3), erythrodermic MF (n = 1), poikilodermatous MF (n = 2) and posttransplant lymphoproliferative disorder (CD8+ MF with gamma/delta phenotype after renal transplantation) (n = 1). One patient had MF concurrent with lymphomatoid papulosis. The group of CBCL comprised six cases (40%) of PCMZL and PCFCL each, 20% (n = 3) were classified as PCLBCL, LT. Conclusion This study for the first time provides data on the distribution of PCL clinicopathologic variants and stages according to the latest classification and staging systems in an Austrian referral centre.

Primary cutaneous lymphoma in Argentina: a report of a nationwide study of 416 patients

“…The data presented here also show that MF was the most common PCL subtype (75.6%). This finding is in accordance with MF rates observed in China (61%) and Iran (87%) but contrasts significantly with MF rates observed in the US (38%), certain European countries (55% in Austria, 51.2% in France, and 41.2% in Switzerland, among others), and South Korea (30.5%) (Table ).…”

“…It is known that cutaneous lymphoma is a disease of the elderly with slightly higher prevalence in men than in women . In Iran, in contrast, the male‐to‐female ratio for PCL was 1:1.2, and the median age at diagnosis was 36 years.…”

“…Within CTCLs, mycosis fungoides (MF) is the most common subtype, with a relative frequency of 44–59% . However, the relative frequencies of PCL subtypes vary among countries on different continents . Moreover, larger geographical variation in the prevalence of rarer forms of lymphoma can be observed, and this may reflect differences in disease pathogenesis.…”

Aggressive Rare T-cell Lymphomas with Manifestation in the Skin: A Monocentric Cross-sectional Case Study

“…However, in the skin, approximately 75% of all cutaneous lymphoma are of T-cell (approximately 65%) and NK-cell (approximately 10%) origin (1)(2)(3)(4)(5). Most of the T-cell lymphomas manifesting in the skin are primary cutaneous, i.e.…”

Disfiguring facial mycoses – a diagnostic and therapeutic challenge

“…In conclusion, in our study, the IR of pCL was higher than previous reports and subtypes distribution a little different. [6][7][8][9][10] An increase in the number of cases around the world, the addition of cases of new entities, improvement in pathology diagnosis techniques, ageing of population and idiosyncratic factors in our population may be responsible. Beyond the burn: psychosocial factors as well as inflammation moderate depression in psoriasis…”