LQCL2508 - CMLE - T-cell Large Granular Lymphocytic Leukemia-LQCL2508

LQCL2508 - Educational Activity

Pdf Summary

The document centers on hematology and specifically on T-cell large granular lymphocytic leukemia (T-LGLL), authored by Wei J Wang, MD, and Jie Xu, MD Ph.D., at MD Anderson Cancer Center, University of Texas. The learning objectives include understanding the morphological and diagnostic characteristics of T-LGLL, knowing its immunophenotypic and genetic traits, and discussing prognosis and treatment.<br /><br />The case in focus describes an 84-year-old man with anemia and mild neutropenia, revealing hematological abnormalities and the presence of T-LGL in peripheral blood and bone marrow, leading to a T-LGLL diagnosis. The diagnosis is supported by specific immunophenotypes and genetic mutations, including STAT3.<br /><br />T-LGLL is depicted as a rare, indolent disorder, more prevalent in Asia, primarily affecting the elderly with a median diagnostic age of 60. Clinical symptoms often involve anemia and neutropenia, while associated conditions may include autoimmune diseases and splenomegaly. Diagnostic markers include specific T-cell immunophenotypes and genetic mutations, with treatment often involving immunosuppressive therapy as there is no standard therapy established.<br /><br />The document underscores the challenges of diagnosing T-LGLL due to its overlap with benign reactive processes. Flow cytometry and genetic studies are pivotal in distinguishing T-LGLL through identifying specific T-cell markers and mutations.<br /><br />In conclusion, T-LGLL is an indolent yet typically manageable disorder, with most patients requiring treatment for anemia or neutropenia. The document rounds off with reference material and an applied quiz about the disease's characteristics, diagnosis, and clinical presentations.

Keywords

T-cell large granular lymphocytic leukemia

hematology

T-LGLL

immunophenotypes

genetic mutations

STAT3

anemia

neutropenia

immunosuppressive therapy

flow cytometry