T-cell prolymphocytic leukemia: an aggressive T cell malignancy with frequent cutaneous tropism

J Am Acad Dermatol. 2006 Sep;55(3):467-77. doi: 10.1016/j.jaad.2006.04.060.


Background: T-cell prolymphocytic leukemia (T-PLL), formerly categorized as T-cell chronic lymphocytic leukemia, is a rare and aggressive hematologic malignancy. Although the skin is characteristically involved, it is not a well-recognized entity in the dermatologic literature.

Methods: Six cases of cutaneous T-PLL are presented from a clinical, light microscopic, and phenotypic perspective.

Results: The patient population comprised 2 women and 4 men, with a mean age of 69.8 years. The disease was associated in all with skin involvement with facial preference; edema, purpura, and lesional symmetry were characteristic. The skin biopsies demonstrated a largely non-epidermotropic angiocentric lymphocytic infiltrate with accompanying hemorrhage. The cells showed irregular- to reniform-shaped nuclei with small nucleoli and eosinophilic rims of cytoplasm. Phenotypic studies revealed three prevailing profiles: CD4 dominant in 4, CD8 dominant in one, and co-expression of CD4 and CD8 in one. CD3 loss was seen in one case. All expressed T-cell leukemia 1 (TCL-1) and CD7; cutaneous lymphocyte antigen expression was discernible in a dot-like perinuclear array. All cases tested excluding one expressed TCL-1 and CD52. In two cases tested, T-cell receptor beta rearrangements were observed. Cytogenetic studies demonstrated a paracentromeric chromosome 14 inversion. Polysomy 8 and MYC amplification was seen in one case, manifesting an aggressive clinical course. Four patients died from their disease within 18 months of diagnosis.

Limitations: Cytogenetic MYC amplification, FISH, and TCR beta studies were conducted on each of 2 cases, respectively, due to limitations of tissue block samples and/or peripheral blood. cMYC translocation studies were conducted on 3 of the 6 cases, again due to limitations imposed by the tissue samples on the cases. The last case was recently diagnosed and, therefore, long-term follow-up is not possible.

Conclusion: T-PLL is a distinctive post-thymic T-cell malignancy with frequent cutaneous tropism. A diagnosis is possible in almost all cases based on characteristic clinical, light microscopic, phenotypic, and cytogenetic features. While a chromosome 14 inversion is highly characteristic, additional inherent cytogenetic differences, such as trisomy 8 with CMYC over-amplification, may account for some case to case variation in clinical course.

MeSH terms

  • Aged
  • Aged, 80 and over
  • Aneuploidy
  • Antigens, CD / metabolism
  • Antigens, Neoplasm / metabolism
  • CD4-Positive T-Lymphocytes / pathology
  • CD52 Antigen
  • CD8-Positive T-Lymphocytes / pathology
  • Cytogenetic Analysis
  • Face
  • Female
  • Gene Amplification
  • Gene Rearrangement
  • Glycoproteins / metabolism
  • Humans
  • In Situ Hybridization, Fluorescence
  • Leukemia, Prolymphocytic / genetics
  • Leukemia, Prolymphocytic / metabolism
  • Leukemia, Prolymphocytic / mortality
  • Leukemia, Prolymphocytic / pathology*
  • Leukemia, Prolymphocytic, T-Cell / genetics
  • Leukemia, Prolymphocytic, T-Cell / metabolism
  • Leukemia, Prolymphocytic, T-Cell / mortality
  • Leukemia, Prolymphocytic, T-Cell / pathology*
  • Male
  • Middle Aged
  • Phenotype
  • Proto-Oncogene Proteins / metabolism
  • Proto-Oncogene Proteins c-myc / genetics
  • Receptors, Antigen, T-Cell, alpha-beta / genetics
  • Skin / pathology*


  • Antigens, CD
  • Antigens, Neoplasm
  • CD52 Antigen
  • CD52 protein, human
  • Glycoproteins
  • MYC protein, human
  • Proto-Oncogene Proteins
  • Proto-Oncogene Proteins c-myc
  • Receptors, Antigen, T-Cell, alpha-beta
  • TCL1A protein, human