What are the genetic features of cutaneous T-cell lymphoma (CTCL)?

Updated: Aug 15, 2018
  • Author: Lauren C Pinter-Brown, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Genetic features show T-cell receptor genes to be clonally rearranged in persons with mycosis fungoides, granulomatous slack skin syndrome, or Sézary syndrome. Demonstration of clonal T cells in the peripheral blood is an important diagnostic criterion that allows differentiation between Sézary syndrome and benign forms of erythroderma. [3]

Recurrent chromosomal translocations are not detected in persons with Sézary syndrome, but complex karyotypes are common, as is a consistent pattern of identical chromosomal abnormalities in Sézary syndrome as seen in mycosis fungoides. [79]

The neoplastic T cells in adult T-cell leukemia/lymphoma express a CD3+, CD4+, CD8- phenotype, with CD25 being highly expressed. [42] T-cell receptor genes are clonally rearranged; clonally integrated HTLV-1 genes are present and are useful in differentiating between chronic or smoldering variants of adult T-cell leukemia/lymphoma and classic mycosis fungoides or Sézary syndrome.

Panniculitis-like T-cell lymphoma shows an alpha/beta+, CD3+, CD4-, CD8+ T-cell phenotype, with expression of cytotoxic proteins. [29] CD30 and CD56 are not expressed. Neoplastic T cells do show clonal T-cell receptor gene rearrangements, although neither specific genetic abnormalities nor EBV has been identified.

Nasal-type extranodal NK/T-cell lymphoma has neoplastic cells that express CD2, CD56, and cytoplasmic CD3.


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