Hepatosplenic T cell lymphoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]

Overview

Genetics

  • Genes involved in the pathogenesis of hepatosplenic T cell lymphoma include:
  • 2-5 copies of i(7)(q10)
  • Isochromosome 7q present
  • Numerical and structural aberrations of the second chromosome 7
  • Rearrangement TRG@, TRB@ genes
  • T-cell receptor (TCR) Gamma/Delta genes are clonally rearranged
  • Trisomy 8

Immunophenotype

The immunophenotype for hepatosplenic T-cell lymphoma is a post-thymic, immature T-cell.

Status Antigens
Positive CD3, TCRδ1, TIA-1, granzyme M, multiple killer immunoglobulin-like receptors (KIR) isoforms
Negative CD4, CD5, CD8, granzyme B, perforin, CD94


Sites of Involvement

The spleen and liver are always involved, with bone marrow involvement frequently present. Nodal involvement is exceedingly rare.[3] Jaffe E.S., Harris N.L., Stein H., Vardiman J.W. (eds): World Health Organization Classification of Tumors. Pathology and Genetics of Tumours of Haemopoietic and Lymphoid Tissues. IARC Press: Lyon 2001 </ref>

Microscopic Pathology

The neoplastic cells in this disorder show a monotonous appearance, with a small amount of cytoplasm and inconspicuous nucleoli.

Spleen and liver

This disease shows a distinct sinusoidal pattern of infiltration which spares the splenic white pulp and hepatic portal triads.

Bone marrow

While the bone marrow is commonly involved, the detection of the neoplastic infiltrate may be difficult due to diffuse, interstitial pattern. Immunohistochemistry can aid in the detection of this lymphoma.

Peripheral blood

Cells of a similar morphology observed in solid organs are observed in peripheral blood.



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