Lymphoma and plasma cell neoplasms
T / NK cell disorders
Hepatosplenic T cell lymphoma

Author: Dragos Luca, M.D.(see Authors page)

Revised: 3 April 2017, last major update August 2011

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

PubMed Search: Hepatosplenic T cell lymphoma

Cite this page: Hepatosplenic T cell lymphoma. PathologyOutlines.com website. http://pathologyoutlines.com/topic/lymphomanonBhepatosplenic.html. Accessed August 23rd, 2017.
Definition / general
Epidemiology
  • Rare, < 1% of all non Hodgkin lymphomas
  • Adolescents and young adults, median age ~35 years
  • Male predominance
Sites
  • Spleen, liver, bone marrow
  • Usually no significant nodal involvement
  • Peripheral blood involvement late in disease; may have blastic transformation
Etiology
Clinical features
  • Hepatosplenomegaly, B symptoms, moderate anemia, marked thrombocytopenia, leukopenia, no lymphadenopathy
  • Peripheral blood involvement uncommon at presentation
Case reports
Prognosis and treatment
  • Aggressive course with relapse in the vast majority of cases
  • Median survival < 2 years
  • Active agents: platinum-cytarabine and 2’-deoxycoformycin
Postulated Normal Counterpart
  • Peripheral γδ (or less commonly αβ) cytotoxic memory T cells of the innate immune system
Gross description
  • Large spleen with diffuse red pulp involvement; no discrete lesions
  • Diffusely enlarged liver
Microscopic (histologic) description
  • Lymphoid infiltrates in splenic red pulp (cords and sinuses), white pulp atrophy, hepatic sinusoids and bone marrow sinuses
  • Intermediate sized monotonous tumor cells with scant to moderate pale / clear cytoplasm, round / oval nuclei, slightly dispersed / loosely condensed chromatin, inconspicuous nucleoli
  • Cytologic atypia with large / blastic cells may occur, especially with disease progression
  • Bone marrow aspirate smears contain malignant cells resembling blasts, occasionally with fine cytoplasmic granules; display sinusoidal pattern in core biopsies
Microscopic (histologic) images

Images hosted on other servers:

Liver and spleen

CD3 epsilon and TIA1

Various images

Liver: H&E, CD3, CD20

Positive stains
  • CD2, CD3, CD7, CD16 (sometimes), CD56 (usually) or CD57, TIA1, granzyme M, Fas ligand, aberrant coincident expression of multiple KIR isoforms
  • TCRδ1, Vδ1 (γδ variant) or TCRαβ, βF1 (αβ variant)
Negative stains
Molecular / cytogenetics description
  • TRG@ (γδ variant) or TRB@ (αβ variant) gene rearrangements
  • Nonproductive TRB@ gene rearrangements reported in some γδ cases
  • Isochromosome 7q (most cases), trisomy 8 (Am J Clin Pathol 2001;116:410)
  • Also multiple copies of i(7)(q10), aberrant second chromosome 7, ring 7, loss of sex chromosome
Molecular / cytogenetics images

Images hosted on other servers:

PCR for TCRγ, Image 3