SITE SEARCH


Clinical Pathological Correlations > Mycosis Fungoides > Diagnosis and Discussion

The patient is a 31 year old man with a history psoriasiform dermatitis and chronic folliculitis.

He has been treated with directed UV irradiation and Targretin. He now presents with progressive skin lesions on his left eyebrow, left cheek, ear and postauricular space.









Diagnosis:

The diagnosis is Mycosis Fungoides with marked Folliculotropism (ref:1,4). Immunohistochemistry showed CD2, CD3 and CD5 positivity. There was reduced CD7 expression and scattered CD8, CD20 and CD56 positive cells. A review of recent literature shows that a GeneScan analysis of γ-chain T-cell receptor rearrangement was more effective than the traditional gel electrophoresis analysis 84% vs. 67% in MF patients (ref:2,3). There is also a high prevalence of oligoclonal TCRγ gene rearrangement with temporary dominance of transient clones (ref:5). Multiple biopsies may be required to identify the pathologic clone.

Reference List

  1. Willemze Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, Ralfkiaer E, Chimenti S, Diaz-Perez JL, Duncan LM, Grange F, Harris NL, Kempf W, Kerl H, Kurrer M, Knobler R, Pimpinelli N, Sander C, Santucci M, Sterry W, Vermeer MH, Wechsler J, Whittaker S, Meijer CJ. WHO-EORTC classification of lymphomas. Blood. May 15;105(10):3768-85.
  2. Vega F, Medeiros LJ, Jones D, Abruzzo LV, Lai R, Manning J, Dunmire V, Luthra R. A novel four-color PCR assay to assess T cell receptor gamma gene rearrangements in lymphoproliferative lesions. Am J Clin Pathol 2001;116: 17-24.
  3. Ponti R, Fierro M, Quaglino P, Lisa B, di Celle Paola F, Michela O, Paolo F, Comessatti A, Novelli M, Bernengo MG. TCRγ-chain gene rearrangement by PCR-based genescan: diagnostic accuracy immprovement and clonal heterogeneity analysi in multiple cutaneous T-cell lymphoma samples. J Investigative Dermatology 2008; 128: 1030-1038.
  4. Smoller BR. Mycosis fungoides:what do/do not we knowγ J Cutan Pathol 2008; 35: 35-39.
  5. Rubben A, Kempf W, Kadin ME, Zimmermann DR, Burg G. Multilineage progression of genetically unstable tumor subclones in cutaneous T cell lymphomas. Exp Dermatol 2004; 153: 565-73.