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Letter to the Editor

Prognosis of Lymphomatoid Papulosis

Department of Dermatology, Innsbruck Medical University, Innsbruck, Austria

Correspondence: Matthias Schmuth, M.D., Department of Dermatology, Anichstraße 35, A-6020 Innsbruck, Austria. Telephone: 43-512-504-81472; Fax: 43-512-504-22990; e-mail: matthias.schmuth{at}uibk.ac.at

Received May 15, 2006; accepted for publication July 10, 2006.

Because of its characteristic waxing and waning course, lymphomatoid papulosis (LyP) was previously considered a pseudolymphomatous inflammatory process. However the recent World Health Organization–European Organization for Research and Treatment of Cancer (WHO-EORTC) classification grouped LyP among the indolent cutaneous lymphomas [1]. The rationale for classifying LyP as a cutaneous lymphoma is its association with other lymphoproliferative disorders, particularly the progression of LyP into frank lymphoma, that is, Hodgkin’s disease, cutaneous T-cell lymphoma, or anaplastic large cell lymphoma.

In their article "Clinical Mimics of Lymphoma," Brown and Skarin [2] report a rate of progression of LyP to frank lymphoma of 20%–80%. The high variability in these numbers prompted us to reassess the rate of progression in our patient cohort and in cases reported in the literature, in particular because of the therapeutic consequences involved. Although it seems clear that undue aggressive treatment must be avoided, a high advancing rate of the disorder should entail a therapeutic approach aimed at halting the disease process. In contrast, a low rate of progression would be in favor of a "wait and see" approach.

A retrospective analysis of our cohort (11 female, 10 male; median age, 47 years; range, 6–77 years) revealed that the absolute frequency of LyP preceding lymphoma was 2 of 21 patients (9.5%) (Table 1). In comparison, absolute frequencies between 5% and 24% have been reported in the literature [3–10] (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). Therefore, the cumulative risk for progression over time may represent a more relevant basis for therapeutic decisions. Yet estimates of the cumulative risk for progression of LyP to lymphoma vary greatly in the literature. For instance, Bekkenk et al. [7] reported cumulative risks in a cohort of 118 LyP patients of 2%, 4%, and 12% at 5, 10, and 15 years after diagnosis of LyP, respectively. In contrast, Cabanillas et al. [8], in a cohort of 21 patients, reported a much higher risk, with rates of approximately 14%, 28%, and >80% after 5, 10, and 15 years, respectively. While the results from the latter study have been attributed to a selection bias that may have led to an overestimation of the risks in that study, nevertheless, other reports also suggested higher cumulative risks. 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 Kaplan-Meier 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 el-Azhary 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.

	DISCLOSURE OF POTENTIAL CONFLICTS OF INTEREST

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The authors indicate no potential conflicts of interest.

	ACKNOWLEDGMENT

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We are indebted to Drs. Karl-Peter Pfeiffer and Hanno Ulmer for advice on statistical analysis.

	REFERENCES

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7. Bekkenk MW, Geelen FA, van Voorst Vader PC et al. Primary and secondary cutaneous CD30⁺ lymphoproliferative disorders: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment. Blood 2000;95:3653–3661.[Abstract/Free Full Text]
8. Cabanillas F, Armitage J, Pugh WC et al. Lymphomatoid papulosis: a T-cell dyscrasia with a propensity to transform into malignant lymphoma. Ann Intern Med 1995;122:210–217.[Abstract/Free Full Text]
9. Sanchez NP, Pittelkow MR, Muller SA et al. The clinicopathologic spectrum of lymphomatoid papulosis: study of 31 cases: J Am Acad Dermatol 1983;8:91–94.
10. el-Azhary RA, Gibson LE, Kurtin PJ et al. Lymphomatoid papulosis: a clinical and histopathologic review of 53 cases with leukocyte immunophenotyping, DNA flow cytometry and T-cell receptor gene rearrangement studies. J Am Acad Dermatol 1994;30:210–218.[Medline]
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13. Schmuth M, Topar G, Illersperger B et al. Therapeutic use of interferon-alpha for lymphomatoid papulosis. Cancer 2000;89:1603–1610.[CrossRef][Medline]

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