RESUMO
Primary cardiac lymphoma (PCL), defined as a lymphoma clinically mimicking cardiac disease, with the bulk of the tumor located intrapericardially, is extremely rare in immunocompetent patients. Clinical manifestations vary depending on sites of involvement in the heart and include chest pain, arrhythmias, pericardial effusion, and heart failure. Diagnosis is often difficult and may require invasive procedures; in some cases, diagnosis is not made until autopsy. Histologically, nearly all cases of PCL reported thus far have been of B-cell origin. In this report, we describe a case of PCL of T-cell origin in an adult immunocompetent patient, the second reported in the literature to the best of our knowledge, and provide a brief overview of the features of previously published PCL cases.
Assuntos
Neoplasias Cardíacas/patologia , Linfoma de Células T/patologia , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biomarcadores Tumorais/sangue , Biópsia , Bleomicina/administração & dosagem , Ciclofosfamida/administração & dosagem , Erros de Diagnóstico , Doxorrubicina/administração & dosagem , Dispneia/etiologia , Evolução Fatal , Feminino , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/tratamento farmacológico , Humanos , Imunofenotipagem , L-Lactato Desidrogenase/sangue , Leucovorina/administração & dosagem , Linfoma de Células T/complicações , Linfoma de Células T/diagnóstico , Linfoma de Células T/tratamento farmacológico , Imageamento por Ressonância Magnética , Metotrexato/administração & dosagem , Pessoa de Meia-Idade , Proteínas de Neoplasias/sangue , Células-Tronco Neoplásicas/química , Células-Tronco Neoplásicas/patologia , Pericardite/diagnóstico , Prednisona/administração & dosagem , Linfócitos T/química , Linfócitos T/patologia , Taquicardia/etiologia , Cirurgia Torácica Vídeoassistida , Vincristina/administração & dosagem , Viroses/diagnósticoRESUMO
Pfeifer-Weber-Christian's panniculitis is a rare syndrome characterized by fever, arthralgias, fatigue and recurrent nodular panniculitis. It has been associated with pancreatic diseases, trauma, connective tissue diseases, alpha-1-antitrypsin deficiency, systemic lupus erythematosus, infections, lymphoproliferative diseases and neoplasias. We report the case of a 43-year-old obese male patient who presented with asthenia, arthralgias, intermittent fever, skin erythema and a large hard-elastic tumor of the right calf. Laboratory analysis revealed increased values of the immunophlogosis parameters and positivity for serum antinuclear antibodies. Surgical drainage of the abscess-like tumor mass, revealed leakage of a sterile, subflavious, oily and thick liquid; a skin biopsy showed intra and perivascular infiltration by neutrophils, diagnostic for leukocytoclastic vasculitis. Treatment with prednisone induced clinical improvement and normalization of the laboratory data. The clinical picture, laboratory data and efficacy of prednisone therapy confirmed that the patient developed Pfeifer-Weber-Christian's panniculitis in the clinical setting of an antinuclear antibody-positive leukocytoclastic vasculitis.