Hepatosplenic T-Cell Lymphoma in Patient With Crohnʼs Disease Receiving Immunomodulator Therapy: A Case Report

Abstract

Hepatosplenic T-cell lymphoma (HSTCL) is a rare and fatal type of Non-Hodgkin Lymphoma characterized by extranodal infiltration and proliferation of malignant T-cells within the liver, spleen and bone marrow. There has been an association with treatment of inflammatory bowel disease with tumor necrosis factor and thiopurines reported in the literature, but no definitive risk has been established. The patient is a 51 year old man who was initially evaluated in hepatology clinic for hepatitis. He had a history of Crohn's disease for which he had undergone ileocolic resection and was well controlled on a regimen of adalimumab and 6-MP. A few months prior, he had been seen at outside hospital for fever and malaise and found to have elevated liver chemistries with total bilirubin of 3.7, alkaline phosphatase 365, AST 510 and ALT 621; ultrasound showed thickened gallbladder wall and mild splenomegaly. Liver MRI a few months later showed hepatomegaly, small benign portacaval lymph nodes and mild splenomegaly. He underwent liver biopsy that was consistent with HSTCL. PET-CT revealed bone marrow and lymph node involvement. He was started on CHOEP with plans for allogeneic stem cell transplant for Stage IVB HSTCL. First cycle was completed inpatient and he was discharged to complete treatment of C. difficile infection on PO flagyl and vancomycin. His treatment course was complicated by tumor lysis syndrome, ESBL bacteremia and altered mental status, likely sepsis induced. He was re-admitted and started on broad spectrum antibiotics for sepsis of unknown origin. During the admission, he had acute liver injury with peak AST 411, ALT 129, and ALP 298, direct bilirubinemia (peak total bilirubin 42, direct bilirubin peaked at 32), coagulopathy (INR peak 4.1) complicated by GIB. He underwent repeat liver biopsy given concern for CHOEP resistance, which showed recurrent/residual HSTCL. He was eventually transferred to medical ICU service given multiorgan failure requiring intubation and initiation of dialysis. Patient was transitioned to comfort care and expired before additional therapy could be initiated. Given the rarity of the disease, it is difficult to identify patients at increased risk for HSTCL or causal relationships between medication exposures and HSTCL. It is our hope that this case report will increase awareness of this disease and facilitate further research to identify at risk patients.