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Related Experiment Videos

T-small lymphocyte disorders.

N L Bartlett1, D L Longo

  • 1Department of Internal Medicine, Washington University School of Medicine, St Louis, MO 63110-1093, USA.

Seminars in Hematology
|May 13, 1999
PubMed
Summary
This summary is machine-generated.

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Small T-cell disorders, including T-cell chronic lymphocytic leukemia (CLL)/prolymphocytic leukemia (PLL), large granular lymphocyte (LGL) leukemia, and mycosis fungoides (MF), are rare lymphoproliferative diseases. While generally indolent, these conditions are not curable.

Area of Science:

  • Hematology
  • Oncology
  • Immunology

Background:

  • Small T-cell disorders constitute less than 2% of lymphoproliferative diseases.
  • These rare conditions include T-cell chronic lymphocytic leukemia (CLL)/prolymphocytic leukemia (PLL), large granular lymphocyte (LGL) leukemia, and mycosis fungoides (MF).

Purpose of the Study:

  • To summarize the clinical characteristics, cytogenetics, and prognoses of various small T-cell disorders.
  • To differentiate the aggressive nature of T-PLL and T-CLL from the generally indolent course of T-LGL leukemia and mycosis fungoides.

Main Methods:

  • Review of clinical features, laboratory findings, and cytogenetic abnormalities associated with T-cell lymphoproliferative disorders.
  • Analysis of survival rates and prognostic factors for each specific T-cell disorder.

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Main Results:

  • T-cell prolymphocytic leukemia (T-PLL) and T-cell chronic lymphocytic leukemia (T-CLL) exhibit aggressive clinical courses with poor median survival, often linked to chromosome 14 abnormalities.
  • T-cell large granular lymphocyte (LGL) leukemia is a clonal cytotoxic T-cell disorder with variable prognosis, generally good survival if neutropenia is managed, but poor outcomes in rare aggressive NK-phenotype subsets.
  • Mycosis fungoides (MF), a cutaneous T-cell lymphoma, follows a chronic, decades-long course, with mortality primarily due to infection.

Conclusions:

  • Small T-cell disorders are a heterogeneous group of rare lymphoid neoplasms.
  • While T-PLL and T-CLL are aggressive, T-LGL leukemia and MF typically follow more indolent, albeit incurable, disease trajectories.
  • Prognosis varies significantly among these disorders, influenced by specific subtypes, clinical features, and cytogenetic findings.