ABCL-407: Daratumumab, Lenalidomide, and Dexamethasone (DRD), an Active Regimen in the Treatment of Sirolimus-Associated Plasmablastic Lymphoma (PBL) in the Setting of Gorham's Lymphangiomatosis: A Case Report

Abstract

Introduction/Background: Plasmablastic lymphoma (PBL) is a rare variant of diffuse large cell B-cell lymphoma characterized by an aggressive course with a poor overall survival. Gorham-Stout Disease (GSD) is a rare skeletal condition of unknown etiology characterized by progressive bone loss and non-malignant proliferation of vascular and lymphatic channels within the affected bone. There is currently no standard of care treatment for either disease. We present a case of PBL induced by sirolimus in a patient with GSD who achieved complete response to treatment with daratumumab, lenalidomide, and dexamethasone (DRD). Case Presentation: A 23-year-old Hispanic female with past medical history of GSD manifesting as lymphedema of left upper extremity, resorption of the left clavicle, and scapular head and humerus, as well as history of chylothoraces, was treated with sirolimus and octreotide. She was admitted for hypoxic respiratory failure requiring intubation. A CT scan showed diffuse lymphadenopathy and a large mediastinal mass. Biopsy showed plasmablastic lymphoma. HIV, EBV, and HHV8 were negative. Sirolimus was discontinued. Dose-adjusted V-EPOCH was initiated but complicated by neutropenic fever and small bowel obstruction. Follow-up CT showed progression of the disease, and the patient was started on DRD. A repeat PET-CT scan after two cycles showed improvement or resolution of previously hypermetabolic lesions and lymph nodes, consistent with complete response (Deauville 2). She underwent evaluation for stem cell transplant but was not a candidate. She continued dose-adjusted DRD but relapsed at 8 months and expired with a total overall survival of 12 months. Discussion: PBL is a difficult condition to treat with a poor overall survival of approximately 15 months and no official recommended treatment regimens at this time. V-EPOCH is often utilized. Our patient had multiple complications while undergoing V-EPOCH. Given the strong CD38+ expression and reported efficacy of lenalidomide in PBL, as well as its antiangiogenic effect for GSD, DRD was selected for treatment in our patient. To our knowledge, this is the first case of PBL treated with DRD achieving complete response. Conclusion: DRD is an active regimen in PBL. Clinical investigations of a DRD regimen in PBL in conjunction with other agents to improve both depth and durability of response is warranted. Plasmablastic lymphoma (PBL) is a rare variant of diffuse large cell B-cell lymphoma characterized by an aggressive course with a poor overall survival. Gorham-Stout Disease (GSD) is a rare skeletal condition of unknown etiology characterized by progressive bone loss and non-malignant proliferation of vascular and lymphatic channels within the affected bone. There is currently no standard of care treatment for either disease. We present a case of PBL induced by sirolimus in a patient with GSD who achieved complete response to treatment with daratumumab, lenalidomide, and dexamethasone (DRD). A 23-year-old Hispanic female with past medical history of GSD manifesting as lymphedema of left upper extremity, resorption of the left clavicle, and scapular head and humerus, as well as history of chylothoraces, was treated with sirolimus and octreotide. She was admitted for hypoxic respiratory failure requiring intubation. A CT scan showed diffuse lymphadenopathy and a large mediastinal mass. Biopsy showed plasmablastic lymphoma. HIV, EBV, and HHV8 were negative. Sirolimus was discontinued. Dose-adjusted V-EPOCH was initiated but complicated by neutropenic fever and small bowel obstruction. Follow-up CT showed progression of the disease, and the patient was started on DRD. A repeat PET-CT scan after two cycles showed improvement or resolution of previously hypermetabolic lesions and lymph nodes, consistent with complete response (Deauville 2). She underwent evaluation for stem cell transplant but was not a candidate. She continued dose-adjusted DRD but relapsed at 8 months and expired with a total overall survival of 12 months. PBL is a difficult condition to treat with a poor overall survival of approximately 15 months and no official recommended treatment regimens at this time. V-EPOCH is often utilized. Our patient had multiple complications while undergoing V-EPOCH. Given the strong CD38+ expression and reported efficacy of lenalidomide in PBL, as well as its antiangiogenic effect for GSD, DRD was selected for treatment in our patient. To our knowledge, this is the first case of PBL treated with DRD achieving complete response. DRD is an active regimen in PBL. Clinical investigations of a DRD regimen in PBL in conjunction with other agents to improve both depth and durability of response is warranted.