Title | T-cell prolymphocytic leukemia: an aggressive T cell malignancy with frequent cutaneous tropism. |
Publication Type | Journal Article |
Year of Publication | 2006 |
Authors | Magro CM, Morrison CD, Heerema N, Porcu P, Sroa N, Deng AC |
Journal | J Am Acad Dermatol |
Volume | 55 |
Issue | 3 |
Pagination | 467-77 |
Date Published | 2006 Sep |
ISSN | 1097-6787 |
Keywords | Aged, Aged, 80 and over, Aneuploidy, Antigens, CD, Antigens, Neoplasm, CD4-Positive T-Lymphocytes, CD52 Antigen, CD8-Positive T-Lymphocytes, Cytogenetic Analysis, Face, Female, Gene Amplification, Gene Rearrangement, Glycoproteins, Humans, In Situ Hybridization, Fluorescence, Leukemia, Prolymphocytic, Leukemia, Prolymphocytic, T-Cell, Male, Middle Aged, Phenotype, Proto-Oncogene Proteins, Proto-Oncogene Proteins c-myc, Receptors, Antigen, T-Cell, alpha-beta, Skin |
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. |
DOI | 10.1016/j.jaad.2006.04.060 |
Alternate Journal | J Am Acad Dermatol |
PubMed ID | 16908353 |
Related Faculty:
Cynthia M. Magro, M.D.