The clinicopathologic spectrum of lymphomatoid papulosis: Study of 31 cases

Prognosis of Lymphomatoid Papulosis

“…Because these reports merely state an average follow-up rather than analyzing the cumulative risk over a defined follow-up period, we present here cumulative risks estimated from these studies by plotting reverse KaplanMeier curves (Table 3). Thus, in two additional studies, the cumulative risks for progression of LyP to lymphoma approached approximately 80% after 30 years: Sanchez et al [9] reported on 31 LyP patients (median age at onset of LyP, 35 years; median follow-up, 9 years), of whom six developed malignant lymphoma after 1-36 years, and elAzhary et al [10] reported on 53 patients (median age at onset, 38 years; median follow-up, 12 years), of whom eight developed lymphoma after 0.5-30 years. These numbers suggest that a loss of patients to follow-up may mislead us to underestimate the true risk for progression of LyP.…”

Polymorphism of the CD30 Promoter Microsatellite Repressive Element Is Associated with Development of Primary Cutaneous Lymphoproliferative Disorders

“…Lymphomatoid papulosis lesions can undergo spontaneous regression or progress to lymphoma and metastasize to regional lymph nodes. The cause of lymphomatoid papulosis is unknown and the potential for clinical progression of this disorder cannot be predicted based on histopathologic criteria as these lesions histologically appear neoplastic in all cases (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12). It would therefore be useful to identify susceptibility and prognostic criteria for this disorder based on the genotypic and molecular characteristics of the atypical cells.…”

“…The male/female ratio was 0.500 among the lymphomatoid papulosis/anaplastic large cell lymphoma patient cohort and 0.541 in the controls. Previous studies have also indicated that there is no age or gender predilection to the development of lymphomatoid papulosis (6,7,9,10,13). To study segregation of structural heterogeneity with the CD30 microsatellite, we also genotyped 20 members of a fourgeneration Caucasian family.…”

“…We identified a microsatellite of the type [(CCAT) [2][3][4][5][6][7][8][9][10][11][12] CCACTTATGCAT] n between positions À1.2 kb and À336 bp of the CD30 promoter, which acts to repress expression of the gene at the transcriptional level (16). The high degree of polymorphism seen in populations of randomly selected individuals at the 30M promoter microsatellite locus (17) suggests that alleles of this microsatellite have the potential to differentially regulate CD30 expression.…”

In Reply

“…In comparison, absolute frequencies between 5% and 24% have been reported in the literature [3][4][5][6][7][8][9][10] (Table 2). However, rather than calculating the absolute frequency in a given LyP patient cohort, an analysis of the course of the disease indicates that there is a considerably increased risk for progression when LyP is followed up for extended time periods (Table 3).…”