Haema 2010; 1(1): 60-64
by J. Kotsiannidis
Department of Haematology, Medical School, Democritus University of Thrace
Abstract
T prolymphocytic leukemia (T-PLL) is a rare hematological disorder of the elderly, comprising only 2% of all small lymphocytic leukemias. Prolymphocyte morphology can be variable and thus immunophenotypic and molecular analyses are needed to confirm the T cell phenotype and the clonal nature of the disease. T-PLL follows an aggressive course and is characterized by marked lymphocytocis and organomegally, whereas lymphadenopathy is usually not prominent. More than 30 years after its first characterization by Galton et al, T-PLL remains an incurable disease, despite the advances in the understanding of its molecular pathogenesis. The introduction of the anti-CD52 monoclonal antibody (alemtuzumab) has improved outcome, but responses are generally short-lived. Likewise, allogeneic transplantation is associated by high morbidity and mortality, presumably because of the advanced patients’ age. It appears that future therapies targeting specific pathways of lymphomagenesis along with the application of less intense transplantation approaches are required to overcome the poor results of conventional therapeutic strategies.