ABSTRACT
Primary cardiac lymphoma (PCL) is a rare and fatal disorder. It may often mimic other common cardiac tumors like cardiac myxoma because of similarities in the clinical presentation. We report a case of PCL of diffuse large B-cell type, in a 38-year-old, immunocompetent male who presented with superior vena cava syndrome that was excised as a myxoma. Histology revealed a large cell population diffusely and strongly expressing CD45, CD20, MUM1/IRF4 and FOXP1 hinting at an activated B-cell (ABC)-like phenotype. After four cycles of Rituximab with CHOP (cyclophosphamide, hydroxydaunorubicin, Oncovin, and prednisolone) the tumor regressed completely but the patient had a relapse and subsequently succumbed to the disease confirming the aggressive nature. The aggressive behavior of PCL may be possibly linked to its ABC-like origin.
Subject(s)
Adult , Antibodies, Monoclonal, Murine-Derived/administration & dosage , Antigens, CD20/biosynthesis , Leukocyte Common Antigens/biosynthesis , Antineoplastic Agents/administration & dosage , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , B-Lymphocytes/immunology , Cyclophosphamide/administration & dosage , Doxorubicin/administration & dosage , Fatal Outcome , Forkhead Transcription Factors/biosynthesis , Gene Expression Profiling , Heart Neoplasms/complications , Heart Neoplasms/diagnosis , Heart Neoplasms/drug therapy , Heart Neoplasms/pathology , Histocytochemistry , Humans , Immunohistochemistry , Interferon Regulatory Factors/biosynthesis , Lymphoma, B-Cell/complications , Lymphoma, B-Cell/diagnosis , Lymphoma, B-Cell/drug therapy , Lymphoma, B-Cell/pathology , Male , Microscopy , Phenotype , Prednisone/administration & dosage , Radiography, Thoracic , Recurrence , Repressor Proteins/biosynthesis , Superior Vena Cava Syndrome/diagnosis , Superior Vena Cava Syndrome/pathology , Tomography, X-Ray Computed , Treatment Outcome , Vincristine/administration & dosageABSTRACT
In addition to CD4+CD25+Foxp3+ regulatory T (T(reg)) cells which protect against autoimmune tissue injury, IL-17-producing CD4+ T (Th17) cells have been recently described and shown to play a crucial role in autoimmune injury. It appears that there is a reciprocal developmental pathway between Th17 and T(reg) cells. Although IL-17 is known to be associated with allograft rejection, the cellular source of IL-17 and the nature of Th17 in the context of allograft rejection remain unknown. In the current study, the dynamics of T(reg) and IL-17-producing cells after syngeneic and allogeneic transplantation were examined using a wild-type murine cardiac transplantation model. Ly6G+ cells were found to produce IL-17 during the early postoperative period and CD8+ as well as CD4+ T cells were also found to produce IL-17 during alloimmune response. Graft-infiltrating Ly6G+, CD4+, and even CD8+ cells were found to express IL-17 highly compared to those in spleen. Although the frequencies of Th17 and T(reg) were found to gradually increase in both syngeneic and allogeneic recipients, Th17/T(reg) ratios were significantly higher in recipients with allograft rejection than in syngeneic recipients. In conclusion, IL-17 is produced by neutrophils during the early postoperative period and subsequently by Th17 and CD8+ T cells during allograft rejection. Th17/T(reg) imbalance is associated with the development of allograft rejection. This study would provide basic information on Th17 biology for future investigation in the field of transplantation.