RESUMEN
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.
Asunto(s)
Adulto , Anticuerpos Monoclonales de Origen Murino/administración & dosificación , Antígenos CD20/biosíntesis , Antígenos Comunes de Leucocito/biosíntesis , Antineoplásicos/administración & dosificación , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Linfocitos B/inmunología , Ciclofosfamida/administración & dosificación , Doxorrubicina/administración & dosificación , Resultado Fatal , Factores de Transcripción Forkhead/biosíntesis , Perfilación de la Expresión Génica , Neoplasias Cardíacas/complicaciones , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/tratamiento farmacológico , Neoplasias Cardíacas/patología , Histocitoquímica , Humanos , Inmunohistoquímica , Factores Reguladores del Interferón/biosíntesis , Linfoma de Células B/complicaciones , Linfoma de Células B/diagnóstico , Linfoma de Células B/tratamiento farmacológico , Linfoma de Células B/patología , Masculino , Microscopía , Fenotipo , Prednisona/administración & dosificación , Radiografía Torácica , Recurrencia , Proteínas Represoras/biosíntesis , Síndrome de la Vena Cava Superior/diagnóstico , Síndrome de la Vena Cava Superior/patología , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Vincristina/administración & dosificaciónRESUMEN
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.