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

@entity4888 @entity1 @entity1071 @entity706 @entity784 @entity1892 @entity12620 @entity74 @entity8548
BACKGROUND: @entity706 ( @entity706 ) are a heterogenous group of rare @entity706 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 @entity706 diagnosed and treated at the Department of Dermatology and Venereology in St. P  lten, 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 @entity706 , @entity4888 ( @entity4888 ) and @entity8548 ( @entity8548 ). @entity1 were diagnosed according to the current WHO-EORTC classification for @entity706 and results were compared with data from European, US and Asian centres. RESULTS: Among 86 @entity1 with @entity706 (age 58.3      17.35  years, mean    SD; @entity1 38%, n  =  33; @entity1 62%, n  =  53) 83% (n  =  71) were classified as @entity784 ( @entity784 ) and 17% (n  =  15) as @entity1892 ( @entity1892 ). Nine @entity1 with @entity784 showed associated @entity74 and malignomas. Among 47 @entity4888 @entity1 following variants were observed: pilotropic @entity4888 (n  =  2), @entity12620 (n  =  1), unilesional @entity4888 (n  =  1), large-cell transformation (n  =  3), erythrodermic @entity4888 (n  =  1), poikilodermatous @entity4888 (n  =  2) and @entity1071 (CD8(+) @entity4888 with gamma/delta phenotype after renal transplantation) (n  =  1). One @entity1 had @entity4888 concurrent with @entity8548 . The group of @entity1892 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 @entity706 clinicopathologic variants and stages according to the latest classification and staging systems in an Austrian referral centre.
