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

Abstract

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.

• Authors: Cynthia M Magro, Carl D Morrison, Nyla Heerema, Pierluigi Porcu, Novie Sroa, April C Deng
• Journal: Journal of the American Academy of Dermatology (J Am Acad Dermatol) Vol. 55 Issue 3 Pg. 467-77 (Sep 2006) ISSN: 1097-6787 [Electronic] United States
• PMID: 16908353 (Publication Type: Journal Article)

Chemical References

• 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

Topics

• 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, metabolism, mortality, pathology)
• Leukemia, Prolymphocytic, T-Cell (genetics, metabolism, mortality, pathology)
• Male
• Middle Aged
• Phenotype
• Proto-Oncogene Proteins (metabolism)
• Proto-Oncogene Proteins c-myc (genetics)
• Receptors, Antigen, T-Cell, alpha-beta (genetics)
• Skin (pathology)