Lymphomatoid papulosis and associated lymphomas: a retrospective case series of 84 patients

Abstract

Referral bias may explain the higher (40%) incidence of lymphoma in this population of LyP patients, compared with the 10-20% incidence commonly cited in the literature. In the subset of patients presenting with LyP alone, only 18% later developed lymphoma. Male patients or patients with prior EBV infection may have a higher risk for developing lymphoma, and some patients improved with treatment of putative infectious triggers.

Frequency and Risk Factors for Associated Lymphomas in Patients With Lymphomatoid Papulosis

“…The need for prolonged follow-up of patients with LyP is also supported by the high 41% frequency of associated lymphomas in the present study. This rate is higher than the commonly reported 10%-20% rates [3,8,9,[11][12][13][14][15]20], and is in accordance with the results from recent studies by Kunishige et al and Liu et al, who reported much higher rates of associated lymphomas (40% and 61%, respectively) [16][17][18]. It is likely that the high proportion of associated lymphomas in these 2 series and the present study (41%) are due to the prolonged follow-up of patients (17.5, 12.3, and 11.3 years respectively), as compared with series that reported lower rates of associated lymphomas [16,18].…”

“…However, in a recent study, only a mixed histological subtype was shown to correlate with increased risk for associated lymphoma in patients with LyP [20]. Older age has also been pointed as a risk factor for association between LyP and another lymphoma in one study [7], but this finding was not confirmed in a more recent and larger one [18].…”

“…The variables with a p value of less than .10 were considered for this multivariate analysis. Variables previously reported to be correlated with presence of an associated lymphoma in the literature (i.e., histological subtype of LyP, sex) [7,18,20] were also systematically incorporated into the model. The positive predictive value (PPV) and negative predictive value (NPV) of TCR clonality were also estimated.…”

“…Diagnosis may precede or be concomitant with that of LyP, or occur during the course of LyP [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] The exact frequency of the association between LyP and another lymphoma remains unclear in the literature. It varies from the commonly cited rates of 10%-20% up to 40% and even 60% in some recent studies [3,[7][8][9][10][11][12][13][14][15][16][17][18][19][20]. This discrepancy led Gruber et al [19] to reassess the cumulative risk of developing an associated lymphoma in patients with LyP on the basis of the largest series [8,[12][13][14].…”

CD8+ Lymphomatoid Papulosis

“…Lymphomatoid papulosis (LyP) is rhythmic paradoxical eruptions of erythematous papules and it has malignant histologic features and a benign clinical course 1 . Immunophenotyping studies of LyP lesions have shown a predominant CD4 expression.…”