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Abstract
Mature T-cell lymphoproliferative disorders are uncommon and can be very challenging diagnoses in hematopathology. Unlike the more common B-cell disorders, in which clonality is often readily discernible by surface immunoglobulin light chain restriction, there is no specific immunophenotypical signature that is diagnostic of a clonal mature T-cell population. The definitive demonstration of T-cell clonality is dependent on molecular biological techniques using PCR or Southern blotting (Gudgin et al., 2005).
Mature T-cell lymphoproliferative disorders comprise a variety of a disease entities which result from the clonal proliferation of post thymic T lymphocytes. Their incidence varies around the world . Overall they are more common in Eastern and rare in Western countries, where they account for 10-15% of lymphoid malignancies. This different distribution may relate to both host and environmental factors. Advances in immunophenotyping and molecular cytogenetics together with a detailed analysis of the cell morphology and histopathology have significantly contributed to a more precise classification of the T-cell neoplasm (Matutes, 2005).
The current World Health Organization (WHO) classification of hematopoietic malignancies defines several types of mature T-cell leukemia including T-cell prolymphocytic leukemia (T-PLL), Sezary syndrome (SS) , T-cell large granular lymphocytic leukemia (T-LGL) and others. These neoplasms can show overlapping features with each other and with T-cell lymphomas involving peripheral blood (Herling et al., 2004).