Liver and intrahepatic bile ducts - tumor
Leukemia / lymphoma
Hepatosplenic gamma-delta T cell lymphoma
Reviewers: Deepali Jain, M.D. (see Reviewers page)
Revised: 9 January 2013, last major update February 2012
Copyright: (c) 2004-2013, PathologyOutlines.com, Inc.
● Rare, < 100 cases reported
● Usually young or middle aged men with massive hepatosplenomegaly and elevated liver function tests, B symptoms (fever, night sweats, weight loss) but no significant nodal involvement, pancytopenia, aggressive clinical course (median survival 8 months)
● Refractory to chemotherapy
● Often occurs after solid organ transplantation, but reduction in immunosuppression does not affect clinical course in Crohn’s disease patients
● 45 year old man with leukemic disease 5 years after renal transplant and continous immunosuppression with cyclosporine A and prednisolone (Hum Pathol 2002;33:253)
● Sinusoidal lymphoid infiltrate of liver (3+ lymphocytes fill / expand sinusoid), spleen and bone marrow by medium sized lymphocytes with condensed chromatin and rim of eosinophilic cytoplasm
● CD2, CD3, CD7, CD16 (50%), CD43, CD45RO, CD56, TIA1, reticulin and T cell receptor gamma-delta chains
● CD4, CD5, CD8, CD57, T cell receptor alpha-beta chain and EBV
● Isochromosome 7q (i7q10 in 2/3), trisomy 8 and Y-
● CMV and EBV have sinusoidal infiltrate but less bulky portal inflammation, hepatocyte ballooning, lobular disarray, higher levels of transaminases
● Drug related hepatitis
End of Liver and intrahepatic bile ducts - tumor > Leukemia / lymphoma > Hepatosplenic gamma-delta T cell lymphoma
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