Bortezomib- and Lenalidomide-Based Treatment of Refractory Plasmablastic Lymphoma

Plasmablastic lymphoma (PBL) is a rare variant of diffuse large B-cell lymphoma for which no optimal treatment has been established and prognosis remains poor. Here, we describe a human immunodeficiency virus-uninfected patient with PBL that was refractory to conventional chemotherapies but was successfully controlled with a bortezomib-based regimen followed by a lenalidomide-based regimen.

A 64-year-old man suffered from nasal bleeding and occlusion. Whole-body computed tomography results revealed a large lesion occupying his nasal cavity. He was diagnosed with PBL based on a tumor biopsy and was treated with two lines of conventional chemotherapy. A dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (EPOCH) regimen and an ifosfamide, carboplatin, and etoposide (ICE) regimen were ineffective, but the bortezomib-based regimen CyBorD (bortezomib, cyclophosphamide orally, and dexamethasone orally) provided a clinical response. Due to peripheral neuropathy, the patient was then treated with a lenalidomide-based regimen (Ld; lenalidomide and dexamethasone). Although a complete response was not achieved, the Ld regimen was tolerated and was continued with a partial response (PR) for over 2 years.

In the present case, PBL that was refractory to conventional chemotherapies responded to the CyBorD regimen and a long-term Ld-based regimen without severe adverse effects. This strategy provided and maintained a PR for over 2 years. Despite not resulting in tumor reduction and only maintaining a PR, continued Ld treatment contributed to long-term survival of the present patient with PBL.