RESUMO
Hepatosplenic T-cell lymphoma (HSTCL) is a rare non-Hodgkin lymphoma with a high mortality rate. Higher incidence is reported in patients with inflammatory bowel disease, specifically in male patients that are younger than 35 years, and have been treated with thiopurine and tumor necrosis factor (TNF)-α inhibitor combination therapy for over 2 years. In this case report we describe a 47-year-old patient with Crohn's disease (CD) who developed HSTCL after having been treated with thiopurine monotherapy for 14 years. To our best knowledge, only eleven cases exist of patients with CD who developed HSTCL while on thiopurine monotherapy. We report the first patient with CD, older than 35 years, who developed HSTCL while on thiopurine monotherapy. This emphasizes that HSTCL risk is not limited to young men receiving both thiopurines and TNF-α inhibitors.
Assuntos
Azatioprina/efeitos adversos , Doença de Crohn/tratamento farmacológico , Imunossupressores/efeitos adversos , Neoplasias Hepáticas/induzido quimicamente , Linfoma de Células T/induzido quimicamente , Neoplasias Esplênicas/induzido quimicamente , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Azatioprina/administração & dosagem , Biópsia , Doença de Crohn/diagnóstico , Doença de Crohn/imunologia , Esquema de Medicação , Evolução Fatal , Hemorragia Gastrointestinal/induzido quimicamente , Humanos , Hospedeiro Imunocomprometido , Imuno-Histoquímica , Imunossupressores/administração & dosagem , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/tratamento farmacológico , Neoplasias Hepáticas/imunologia , Linfoma de Células T/diagnóstico , Linfoma de Células T/tratamento farmacológico , Linfoma de Células T/imunologia , Masculino , Pessoa de Meia-Idade , Mucosite/induzido quimicamente , Tomografia por Emissão de Pósitrons , Fatores de Risco , Neoplasias Esplênicas/diagnóstico , Neoplasias Esplênicas/tratamento farmacológico , Neoplasias Esplênicas/imunologia , Fatores de TempoRESUMO
Three male patients aged between 50 and 70 years were referred with jaundice and weight loss. Imaging showed a pancreatic mass and changes in the calibre of the choledochal or pancreatic duct, suggestive of malignancy. Two patients were operated on. One patient was considered to have an unresectable carcinoma but showed remarkable clinical improvement after steroids were given for his poor condition. In the other patient a resection was performed. Histology showed IgG4-positive plasma cell infiltration without signs of malignancy. Eventually these patients were diagnosed with auto-immune pancreatitis (AIP). In the third patient AIP was considered beforehand and this patient was treated with steroids. He responded quickly both clinically and radiologically. CT imaging showed complete remission of the mass. AIP is a benign inflammatory process which can mimic pancreatic carcinoma. In doubtful cases, a short trial of steroids might be considered.
