Stefanie Porkert, Pamela Mai, Constanze Jonak, Felix Weihsengruber, Klemens Rappersberger, Wolfgang Bauer, Ingrid Simonitsch-Klupp, Markus Raderer, Julia Valencak
DOI: 10.2340/00015555-3746

Systemic monotherapy with rituximab is a well-known treatment approach for primary cutaneous follicle centre lymphoma and primary cutaneous marginal zone lymphoma. Both have excellent prognosis despite high relapse rates. To investigate the long-term effectiveness and clinical outcome of intravenous rituximab at a dose of 375 mg/m2 once weekly, data for 26 patients (17 primary cutaneous follicle centre lymphoma and 9 primary cutaneous marginal zone lymphoma) were analysed retrospectively. Complete remissions occurred in 20 (77%) and partial remissions in 6 patients (23%), demonstrating an overall response rate of 100%. The relapse rate was 52.9% in primary cutaneous follicle centre lymphoma and 88.9% in primary cutaneous marginal zone lymphoma. Ongoing complete remissions after therapy with rituximab were observed in 9 patients (34.6%) with a median progression-free survival of 161 months (13.4 years). These results confirm that intravenous rituximab is an effective and well-tolerated treatment with durable responses in a relevant percentage of patients at a median follow-up of 148 months (12.3 years).

Primary cutaneous follicle centre lymphoma and primary cutaneous marginal zone lymphoma are the second most common primary cutaneous B-cell lymphomas, with high relapse rates, requiring several therapies in the course of disease. Although surgical excision or radiotherapy are recommended first-line treatments, a high proportion of patients present with multifocal disease or extended lesions. In recent decades, intravenous rituximab has been used successfully in primary cutaneous B-cell lymphomas. This study shows that intravenous rituximab is an effective and safe treatment for primary cutaneous follicle centre lymphoma and primary cutaneous marginal zone lymphoma in long-term follow-up.