BACKGROUND: Primary cutaneous diffuse large B-cell lymphoma (PCLBCL) represents a rare subtype among primary cutaneous B-cell lymphoma exhibiting a characteristic genetic background, an aggressive clinical course and a high relapse rate under different therapeutic regimen. Therefore, PCLBCL has a rather restricted prognosis. PATIENTS AND METHODS: Four patients with PCLBCL were treated at our institution with age- and toxicity-adapted first-line immunochemotherapy with rituximab and modified CHOP (cyclophosphamid, vincristin, liposomal doxorubicin, prednisolon). On relapse, the same regimen with R-CHOP or different antineoplastic strategies (radiation, polychemotherapy, immunotherapy, stem cell transplantation) were applied. Toxicity, clinical response and overall survival was documented. RESULTS: Under this regimen, clinical response to modified R-CHOP was achieved in all patients with tolerable toxicity - however, being characterized by a rapid disease progression with inconsistent response towards the subsequent therapeutic armentarium and unsecure impact on overall survival. CONCLUSIONS: So far, it is still unknown, if an extensive multimodal therapy for PBLBCL improves overall survival. Immunochemotherapy with R-CHOP currently represents the most effective treatment.
BACKGROUND: Primary cutaneous diffuse large B-cell lymphoma (PCLBCL) represents a rare subtype among primary cutaneous B-cell lymphoma exhibiting a characteristic genetic background, an aggressive clinical course and a high relapse rate under different therapeutic regimen. Therefore, PCLBCL has a rather restricted prognosis. PATIENTS AND METHODS: Four patients with PCLBCL were treated at our institution with age- and toxicity-adapted first-line immunochemotherapy with rituximab and modified CHOP (cyclophosphamid, vincristin, liposomal doxorubicin, prednisolon). On relapse, the same regimen with R-CHOP or different antineoplastic strategies (radiation, polychemotherapy, immunotherapy, stem cell transplantation) were applied. Toxicity, clinical response and overall survival was documented. RESULTS: Under this regimen, clinical response to modified R-CHOP was achieved in all patients with tolerable toxicity - however, being characterized by a rapid disease progression with inconsistent response towards the subsequent therapeutic armentarium and unsecure impact on overall survival. CONCLUSIONS: So far, it is still unknown, if an extensive multimodal therapy for PBLBCL improves overall survival. Immunochemotherapy with R-CHOP currently represents the most effective treatment.