While DLBCL-CI is an aggressive malignancy, its treatment, particularly in localized disease, should include efforts to remove its underlying inflammatory causes. For example, PAL is a particularly aggressive form of DLBCL-CI. Nonetheless, surgical removal of the pleural tumor effectively treats the few cases in which it is highly localized and of low-grade. Historically, severe cases of PAL have been treated with chemotherapy regimens such as CHOP (i.e. cyclophosphamide, hydroxydoxorubicin, vincristine, and prednisone); Overall survival rates with this regimen have been poor, e.g. ~21% after 5 years. More recently, PAL has been treated with the immunochemotherapy regimen of R-CHOP, i.e. CHOP plus the immuotherpeutic agent, rituximab. Rituximab is a commercial monoclonal antibody that binds to the CD20 cell surface protein on B-cells to thereby<unk> t these cells for attack by the hosts adaptive immune system. The addition of rituximab to chemotherapy regimens such as CHOP has greatly improved the prognosis of most DLBCL variants and modestly improved the outcome in patients with the Epstein–Barr virus-positive<unk><unk>  B-cell lymphoma, not otherwise specified variant of DLBCL. There are too few reports on the treatment of non-PAL forms of DLBCL-CI to make recommendations although the R-CHOP regimen is being used as first-line treatment for severe DLBCL-CI cases that are not PAT. The R-CHOP regiment or similar immunochemotherapeutic regimen (e.g. EPOCH, i.e. rituximab plus etoposide, prednisolone, oncovin, cyclophosphamide, and hydroxydaunorubicin) may prove useful for treating DLBCL-CI. New treatment studies An interventional phase II clinical trial testing the effectiveness and safety of R-CHOP versus R-CHOP plus lenalidomide (unclear mechanism of action) in 570 previously untreated participants with various forms of DLBCL, including DLBCL-CI, has finished recruitment of participants. It has an estimated study completion date of August 3, 2022. A phase II clinical trial is recruiting individuals to study how well nivolumab with or without varlilumab works in treating patients with aggressive B-cell lymphomas, including DLBCL-CI, that have come back, or do not respond, to immunotherapy with either one of these monoclonal antibodies. A phase I clinical trial is recruiting individuals to study the side effects and efficacy of CD19/CD22 chimeric antigen receptor T cells (i.e. T-cells from a donor patent are obtained, engineered to attack cells that express CD19 or CD22, and then injected back into the donor) when given together with chemotherapy in treating patients with DLBCL, including DLBCL-CI, or B-cell acute lymphoblastic leukemia. Only individuals whose neoblastic B-cells express the CD19 cell surface protein are eligible to enter this study.
Diffuse large B-cell lymphoma associated with chronic inflammation
Large B-cell lymphoma arising in HHV8-associated multicentric Castleman's disease
Splenic lymphoma with villous lymphocytes
Germinal center B-cell like diffuse large B-cell lymphoma
Chronic multifocal Langerhans cell histiocytosis
Thymoma-associated multiorgan autoimmunity
Epstein–Barr virus positive diffuse large B-cell lymphoma, not otherwise specified
Lymphoid neoplasms with plasmablastic differentiation