Liver and intrahepatic bile ducts - tumor
Leukemia / lymphoma
Hepatosplenic gamma delta T cell lymphoma

Author: Deepali Jain, M.D. (see Authors page)

Revised: 29 November 2017, last major update February 2012

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Hepatosplenic gamma delta T cell lymphoma[TIAB]

Cite this page: Jain, D. Hepatosplenic gamma delta T cell lymphoma. PathologyOutlines.com website. http://pathologyoutlines.com/topic/livertumorhepatosplenicgammadelta.html. Accessed December 15th, 2017.
Definition / general
  • 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
Case reports
  • 45 year old man with leukemic disease 5 years after renal transplant and continuous immunosuppression with cyclosporine A and prednisolone (Hum Pathol 2002;33:253)
Microscopic (histologic) description
  • 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
Positive stains
Negative stains
Molecular / cytogenetics description
  • Isochromosome 7q (i7q10 in 2/3), trisomy 8 and Y-
Differential diagnosis
  • CMV and EBV have sinusoidal infiltrate but less bulky portal inflammation, hepatocyte ballooning, lobular disarray, higher levels of transaminases
  • Drug related hepatitis