Investigation of Atopy in Primary Cutaneous CD30+ T-Cell Lymphoproliferative Disorders

Background: A link between atopy and primary cutaneous CD30+ lymphoproliferative disorders (CD30CLPD), which encompasses lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large cell lymphoma (pcALCL), has been suggested in the literature. Objective: Investigate whether patients with CD30CLPD have an atopic diathesis. Methods: We reviewed our experience with 157 patients with LyP and 23 patients with pcALCL for: (1) the patients' personal history of seasonal allergic rhinitis, allergic asthma or atopic dermatitis/eczema, (2) a history of these conditions in a first degree family member, and (3) a serum total IgE level that exceeds 100 IU/mL which in adults has been used to signify “probable atopy”. Also recorded were a history of patients' allergic reactions to medications and absolute peripheral blood eosinophil counts. Results: A personal history of at least one atopic disorder was noted for 38% and 17% of patients with LyP and pcALCL, respectively. Allergic rhinitis was the most frequent allergy in both LyP and pcALCL subsets (27% overall) with asthma and eczema reported in 10% or fewer cases. The prevalence of allergic rhinitis in these subsets was significantly lower than the 38.2% prevalence observed for healthy controls derived from the Interlymph project. However, recall bias and unstructured collection of data may contribute to these different results. The frequency of allergic drug reactions, most often to penicillin type drugs, were similar for both LyP and pcALCL subsets (28% overall). The frequency of penicillin reactions for patients with pcALCL was significantly higher than the 11.46% rate reported by Albin (P= 0.01). At least one atopic condition was recorded for a parent, sibling or child in about 20% of patients with either LyP or pcALCL. Multiple atopic conditions in first degree family members occurred in about a third of these cases, in particular for family members of patients with LyP-C. Mean total serum IgE values were significantly increased in patients with LyP and pcALCL compared to published reference values for non-atopic adults in the US and Europe. Between 30 to 36% of patients with LyP type A, LyP type C and pcALCL, but not LyP type B, had total IgE values that exceeded 100 IU/ml. Although not statistically significant, mean IgE levels tended to increase from LyP-B to LyP-A to LyP-C and pcALCL, suggesting a possible role of atypical CD30+ cells in directly stimulating IgE production. Eosinophilia was present in 4% of cases. Conclusion: This study provides evidence that serum total IgE is often increased in patients with CD30CLPD. The IgE> 100 IU/ml threshold for probable atopy was exceeded in about a third of patients with LyP-A, LyP-C and pcALCL, but not LyP-B. However, a link with atopy was not supported by review of the patients' personal and family history that was obtained at the time of the initial examination rather than using a formal questionnaire. Additional studies with measurement of allergen specific IgE antibodies with attention to bacterial superantigens might be more informative.