T-CELL PROLYMPHOCYTIC LEUKEMIA

Course overview

T-PLL (T PROLYMPHOCYTIC LEUKEMIA) IS A MATURE AND AGGRESSIVE T-CELL LEUKEMIAS CHARACTERIZED BY THE PROLIFERATION OF SMALL TO MEDIUM-SIZED PROLYMPHOCYTES THAT SHOW MATURE OR POST-THYMIC T CELL PHENOTYPE. T-PLL INVOLVES PERIPHERAL BLOOD (PB), BONE MARROW (BM), LYMPH NODES, LIVER, SPLEEN, AND SKIN. THE NAME "PROLYMPHOCYTE" IS INACCURATE, AS THE TUMOR CELLS IN THIS DISEASE ARE OF POST-THYMIC T CELL IN ORIGIN. T-PLL INCREASES IN ATAXIA-TELANGIECTASIA (ATM) PATIENTS. WHOLE-GENOME SEQUENCING AND WHOLE-EXOME SEQUENCING HAVE DEMONSTRATED MUTATIONS IN THE FOLLOWING GENES: IL2RG, JAK1, JAK3, STAT5B, EZH2, FBXW10, AND CHEK2. THIS ACTIVITY REVIEWS THE EVALUATION OF T CELL PROLYMPHOCYTIC LEUKEMIA AND IDENTIFIES THE ROLE OF THE INTERPROFESSIONAL TEAM IN MANAGING THIS CONDITION.