ABSTRACT
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.
Subject(s)
Azathioprine/adverse effects , Crohn Disease/drug therapy , Immunosuppressive Agents/adverse effects , Liver Neoplasms/chemically induced , Lymphoma, T-Cell/chemically induced , Splenic Neoplasms/chemically induced , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Azathioprine/administration & dosage , Biopsy , Crohn Disease/diagnosis , Crohn Disease/immunology , Drug Administration Schedule , Fatal Outcome , Gastrointestinal Hemorrhage/chemically induced , Humans , Immunocompromised Host , Immunohistochemistry , Immunosuppressive Agents/administration & dosage , Liver Neoplasms/diagnosis , Liver Neoplasms/drug therapy , Liver Neoplasms/immunology , Lymphoma, T-Cell/diagnosis , Lymphoma, T-Cell/drug therapy , Lymphoma, T-Cell/immunology , Male , Middle Aged , Mucositis/chemically induced , Positron-Emission Tomography , Risk Factors , Splenic Neoplasms/diagnosis , Splenic Neoplasms/drug therapy , Splenic Neoplasms/immunology , Time FactorsABSTRACT
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.
