Retrospective Evaluation of Clinical and Follow-Up Outcomes in Primary Cutaneous CD30+ Lymphoproliferative Disorders

Abstract

This study evaluated the demographic data, clinical characteristics, treatment approaches, and treatment responses of 43 patients with primary cutaneous CD30⁺ lymphoproliferative disorders. Lymphomatoid papulosis (LyP) was characterized by predominantly papular (94.1%) and generalized (70.6%) lesions, while primary cutaneous anaplastic large-cell lymphoma (pcALCL) presented with tumoral (77.8%) and solitary (77.8%) lesions (p<0.001). Common treatments for LyP included methotrexate (response rate: 78.5%), topical corticosteroids (response rate: 83.3%), and phototherapy (response rate: 85.8%), but relapse rates were high. In pcALCL, complete remission was achieved with all treatments, with no relapses after brentuximab vedotin (BV). Secondary malignancies were noted in 20.6% of LyP cases. Both LyP and pcALCL had a 100% 5-year disease-specific survival rate, although two LyP patients (5.9%) died of secondary malignancies. In conclusion, LyP and pcALCL are both indolent lymphomas, with LyP being more prone to relapse. BV is effective for resistant pcALCL. LyP patients need long-term monitoring due to the risk of secondary malignancies.