Lymphomatoid papulosis: Treatment response and associated lymphomas in a study of 180 patients

Background Lymphomatoid papulosis (LyP) is a CD30+ lymphoproliferative disorder, with a self-regressing clinical course and malignant histopathology. Objective The aim of this study was to evaluate characteristics, risk factors, associated malignancies, long-term outcome, and treatment of LyP in a large cohort representing the experience of the MD Anderson Cancer Center. Methods Patient charts and clinical and histopathologic data of 180 patients with LyP were retrospectively assessed. Results A total of 56.7% of patients was men. Histologic subtype A was found in 47.2%, type B in 17.2%, type C in 22.8%, type D in 7.8%, type E in 0.6%, and mixed subtype in 4.4% of the patients. One hundred fourteen lymphomas were observed in 93 patients, with mycosis fungoides (61.4%) and anaplastic large cell lymphoma (26.3%) being the most common forms. Risk factors for development of lymphoma included sex and histologic subtype. Number of lesions and symptom severity were not associated with lymphoma development. Patients with type D were less likely to have lymphomas. Treatment provided symptomatic relief but did not prevent progression to lymphoma. Limitations The limitation of this study is the retrospective study design. Conclusion Patients with LyP are at increased risk of associated lymphomas. Thorough patient counseling is needed and long follow-up periods are required to detect and treat secondary lymphomas.