T-cell prolymphocytic leukemia: a single-institution experience.
BACKGROUND
T-cell prolymphocytic leukemia is an uncommon, aggressive, mature T-cell leukemia characterized by proliferation of T-cell lymphocytes. The recent availability of modern immunophenotypic and molecular tools has allowed a better distinction of this disorder from its B-cell counterpart and other mature T-cell leukemias.


METHODS
The clinical, pathologic, and cytogenetic features of 57 patients with T-PLL who were evaluated at the Department of Leukemia, M. D. Anderson Cancer Center (MDACC) from 1986 to 2004 were examined.


RESULTS
The most common cytogenetic abnormality was inv(14)(q11;q32), which was present in 7 patients. In all 7 patients, this abnormality was associated with other chromosomal aberrations. Patients treated with alemtuzumab at MDACC had a significantly better response rate (P = 0.02) and survival rate (P = 0.002). There were no significant differences in survival based on Tcl-1 expression or different patterns of CD4 and CD8 expression.


CONCLUSIONS
Treatment with alemtuzumab results in higher response rates and a better survival rate in patients with T-cell prolymphocytic leukemia.
Ravandi
Farhad
F
Department of Leukemia, University of Texas M. D. Anderson Cancer Center, Houston, Texas 77030, USA. fravandi@mdanderson.org
O'Brien
Susan
S
Jones
Dan
D
Lerner
Susan
S
Faderl
Stefan
S
Ferrajoli
Alessandra
A
Wierda
William
W
Garcia-Manero
Guillermo
G
Thomas
Deborah
D
Koller
Charles
C
Verstovsek
Srdan
S
Giles
Francis
F
Cortes
Jorge
J
Herling
Marco
M
Kantarjian
Hagop
H
Keating
Michael
M
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000096:biosynthesis*; D015496:CD4-Positive T-Lymphocytes / Q000378:metabolism / Q000473:pathology; D018414:CD8-Positive T-Lymphocytes / Q000378:metabolism / Q000473:pathology; D007446:Chromosome Inversion; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D015463:Leukemia, Prolymphocytic / Q000188:drug therapy / Q000378:metabolism* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011518:Proto-Oncogene Proteins / Q000096:biosynthesis*; D012189:Retrospective Studies
D014408:Biomarkers, Tumor; D011518:Proto-Oncogene Proteins; C090851:TCL1A protein, human