LQCL2507 - CMLE - Hepatosplenic T-Cell Lymphoma-LQCL2507

LQCL2507 - Educational Activity

Pdf Summary

The document is a detailed educational module on Hepatosplenic T-cell Lymphoma (HSTCL), a rare and aggressive form of T-cell lymphoma. The study is authored by medical professionals Xenia Parisi, MD, and Jie Xu, MD, PhD, from the MD Anderson Cancer Center at the University of Texas. The module outlines the learning objectives, clinical history, laboratory challenges, and case conclusions related to HSTCL.<br /><br />Key learning points include the clinical presentation, morphological features visible in blood and bone marrow smears, immunophenotype, differential diagnosis, and prognosis of HSTCL. The document details a case study of a 37-year-old male patient presenting with chest discomfort and shortness of breath. His medical examination revealed mild anemia, elevated lactate dehydrogenase (LDH) levels, splenomegaly, and an anterior mediastinal mass. Subsequent blood tests and bone marrow biopsies identified atypical lymphocytes and an aberrant T-cell population, leading to a diagnosis of HSTCL.<br /><br />HSTCL primarily affects individuals in their forties, though it can occur in different age groups, with a noted male predominance. The lymphoma is derived mostly from gamma-delta T-cells and is associated with immunodeficiency conditions. The section on laboratory challenge emphasizes accuracy in assessing atypical lymphoid cells and details the morphological appearance of HSTCL cells, which may mimic reactive lymphocytes or blasts.<br /><br />Prognosis is generally poor, with a median survival of up to two years, although aggressive treatments may extend it. The case concluded with the patient resisting multiple chemotherapy regimens and eventually succumbing to hemophagocytic lymphohistiocytosis.<br /><br />Furthermore, the document discusses associations with immunodeficiency, the importance of comprehensive clinical and laboratory evaluations for accurate diagnosis, and the typical immunophenotypes and cytogenetic markers observed in HSTCL cases. Lastly, there are educational quizzes provided to assess understanding.

Keywords

Hepatosplenic T-cell Lymphoma

HSTCL

T-cell lymphoma

Xenia Parisi

Jie Xu

clinical presentation

immunophenotype

differential diagnosis

prognosis

immunodeficiency