Liver and intrahepatic bile ducts - tumor
Leukemia / lymphoma
Hepatosplenic alpha beta 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.
● May be clinical variant of gamma-delta T cell lymphoma, but less common (Am J Surg Pathol 2001;25:285)
● Associated with thrombocytopenia, anemia, hepatosplenomegaly, B symptoms, but usually no lymphadenopathy
● Mean age 36 years old, but wide range; female preponderance
● Aggressive clinical course leading to death
● 12 year old boy with hepatosplenomegaly, anemia and thrombocytopenia (Am J Surg Pathol 2001;25:970)
● 16 year old boy with jaundice, splenomegaly, anemia, thrombocytopenia (Am J Surg Pathol 2000;24:459)
● 20 year old man with hepatosplenomegaly, mild anemia and thrombocytopenia (Am J Surg Pathol 2000;24:1027)
● 20 year old woman with S100+ lymphoma (Arch Pathol Lab Med 2003;127:e119)
● Sinusoidal lymphoid infiltrate within liver, splenic red pulp and bone marrow of small-medium sized atypical lymphoid cells with scant / moderate cytoplasm, round / oval nuclei, slightly dispersed chromatin and indistinct nucleoli
● Marked periportal infiltrates in CD57+ cases
H&E, CD45RO and S100
● CD2, CD3, CD8 (61%), CD16 (44%), CD45RO, CD57 (40%), TIA1, reticulin and T cell receptor alpha-beta chains
● CD4, CD5
● Isochromosome 7q
End of Liver and intrahepatic bile ducts - tumor > Leukemia / lymphoma > Hepatosplenic alpha beta T cell lymphoma
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