LQCL2509 - CMLE - T-Prolymphocytic Leukemia-LQCL2509

LQCL2509 - Educational Activity

Pdf Summary

The document is a hematology exercise developed by the MD Anderson Cancer Center and ASCP LabQ, focusing on T-prolymphocytic leukemia (T-PLL). It aims to educate medical professionals on the characteristics, diagnosis, and prognosis of T-PLL. T-PLL is a rare, aggressive form of leukemia characterized by the clonal proliferation of small-to-medium-sized prolymphocytes with a mature T-cell phenotype. Commonly affecting adults over 30, it often involves the peripheral blood, bone marrow, spleen, liver, and lymph nodes, and in some cases, the skin and serosa.<br /><br />Clinical presentation typically includes lymphocytosis, hepatosplenomegaly, and generalized lymphadenopathy. Peripheral blood smear reveals atypical lymphocytes with irregular nuclear contours, basophilic cytoplasm, prominent nucleoli, and cytoplasmic blebs. Flow cytometry and immunohistochemistry identify an aberrant T-cell population expressing markers such as CD2, CD3, CD5, CD7, CD26, and TCL1. Cytogenetic studies often show abnormalities like inv(14)(q11q32) and the TCL1 rearrangement.<br /><br />The average survival time for T-PLL patients is 1-2 years, with the best responses seen following treatment with the monoclonal antibody alemtuzumab (anti-CD52), often necessitating stem cell transplantation for eligible patients. Prognosis is generally poor, though some patients initially present a stable disease course before progressing.<br /><br />Educational objectives include recognizing the morphologic, immunophenotypic, and cytogenetic features of T-PLL, evaluating differential diagnoses (such as other mature T-cell and lymphoid neoplasms), and understanding the disease's prognosis and treatment options. The exercise underscores the importance of medical laboratory scientists in identifying T-PLL through careful examination of peripheral blood smears.

Keywords

T-prolymphocytic leukemia

MD Anderson Cancer Center

ASCP LabQ

diagnosis

prognosis

flow cytometry

immunohistochemistry

alemtuzumab

cytogenetic studies

peripheral blood smear