Human pathology

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cutaneous T-cell lymphomas

CTCL

Cutaneous T-cell lymphoma (CTCL) is a clonally derived lymphoproliferative disorder that preferentially involves the skin. The two major clinical expressions of CTCL, mycosis fungoides (MF) and Sézary syndrome (SS), have poorly understood pathogenesis.

Types

- mycosis fungoides (MF)
- Sezary syndrome (SS)
- primary cutaneous CD30+ lymphoproliferative disorders

  • primary cutaneous anaplastic large cell lymphoma
  • lymphomatoid papulosis
  • borderline lesions

- subcutaneous panniculitis-like T-cell lymphoma
- primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma
- primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma
- cutaneous gamma/delta T-cell lymphoma

Cytogénétique

Chromosome abnormalities, mostly complex karyotypes, are seen in about 50% of patients with MF/SS, and there have only been a few instances of recurrent rearrangements.

- 47% of abnormal karyotype by SKY/FISH (16382449)

  • chromosome 10
  • chromosome 6 (deletion)
  • chromosome 3
  • chromosome 7
  • chromosome 9
  • chromosome 17
  • chromosome 19
  • chromosome 1
  • chromosome 12
  • chromosome 8
  • chromosome 11
  • chromosome 13 (deletion)

- recurrent breakpoints

- pseudodicentric translocation between the short arms of chromosomes 8 - dic(17;8)(p11.2;p11.2) (16382449)

See also

- Tumors

References

- Batista DA, Vonderheid EC, Hawkins A, Morsberger L, Long P, Murphy KM, Griffin CA. Multicolor fluorescence in situ hybridization (SKY) in mycosis fungoides and Sezary syndrome: search for recurrent chromosome abnormalities. Genes Chromosomes Cancer. 2006 Apr;45(4):383-91. PMID: 16382449