Unusually indolent T-cell prolymphocytic leukemia associated with a complex karyotype: is this T-cell chronic lymphocytic leukemia?
T-cell prolymphocytic leukemia (T-PLL) is typically associated with an aggressive clinical course, with a median survival of less than 1 year. We report a case of T-PLL that displays multiple cytogenetic abnormalities, with the most complex subclone having the following karyotype: 47, Y, -X, +8, inv (10) (p12q26), del (11) (p13p15) +marker. However, despite this genetic complexity, the leukemia has behaved in a remarkably indolent manner, with the patient remaining asymptomatic, without therapeutic intervention, for more than 7 years. The unusually benign behavior of this disease calls into question the validity of grouping such cases under the umbrella of T-PLL and warrants a reconsideration of T-cell chronic lymphocytic leukemia (no longer recognized as a distinct disease) as a bona fide diagnostic entity.
Soma
Lorinda
L
Department of Pathology and Laboratory Medicine, University of Pennsylvania Medical Center, 3400 Spruce Street, Philadelphia, PA 19104, USA.
Cornfield
Dennis B
DB
Prager
David
D
Nowell
Peter
P
Bagg
Adam
A
D000368:Aged; D020732:Cytogenetic Analysis; D003937:Diagnosis, Differential; D006801:Humans; D007621:Karyotyping; D015463:Leukemia, Prolymphocytic / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology; D015461:Leukemia, Prolymphocytic, T-Cell / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology; D008297:Male