CD30+ cutaneous lymphoproliferative disorders:: The Stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma

Background: CD30(+) cutaneous lymphoproliferative disorders (CLPDs) include lymphomatoid papulosis, borderline cases of CD30(+) CLPDs, and primary cutaneous anaplastic large cell lymphoma (PCALCL). Prior studies have shown CD30(+) CLPDs have an excellent prognosis. Objective: We sought to present the single-center experience of Stanford University, Stanford, Calif, in the management of CD30(+) CLPDs. Methods: A retrospective cohort analysis of 56 patients with CD30(+) CLPDs treated at our institution was performed. Results: No patients with lymphomatoid papulosis died of disease, and overall survival was 92% at 5 and 10 years. Disease-specific survivals at 5 and 10 years for PCALCL were 85%. Disease-specific survival at 5 years for localized versus generalized PCALCL was 91% versus 50% (P = .31). PCALCL was highly responsive to treatment, but the relapse rate was 42%. In all, 3 patients progressed to extracutaneous stage of disease. No clinical or histologic factors analyzed were predictive of worse outcome in lymphomatoid papulosis and PCALCL. Conclusion. Similar to prior reports from multicenter European groups, the single-center experience at our institution demonstrates CD30(+) CLPDs have an overall excellent prognosis; however, cases of PCALCL with poor outcome do exist.