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Cytokine profile in acute myelofibrosis associated with aggressive large granular lymphocyte leukemia

B I Dalal1, P A Keown, F Paraskevas

  • 1Division of Hematopathology, Vancouver General Hospital, BC, Canada.

American Journal of Hematology
|August 1, 1995
PubMed

Insights

This study details a rare case of acute large granular lymphocyte (LGL) leukemia mimicking acute myelofibrosis (AMF). The T-cell leukemia exhibited specific genetic and cytokine profiles, including high Transforming Growth Factor-beta 1.

Area of Science:

  • Hematology
  • Oncology
  • Immunology

Background:

  • Large granular lymphocyte (LGL) leukemia is a rare lymphoproliferative disorder.
  • Acute myelofibrosis (AMF) is a distinct clinicopathologic entity characterized by bone marrow fibrosis.
  • The co-occurrence of LGL leukemia presenting as AMF is exceptionally uncommon.

Observation:

  • A patient presented with clinical and hematological features suggestive of acute myelofibrosis.
  • Flow cytometry revealed immature T-cells with a specific immunophenotype (CD5+, CD7+, CD16-, CD56-, CD57-, CD41-).
  • Genetic analysis identified monosomy 7 in the leukemic cells.

Findings:

  • The leukemic cells were found to secrete substantial amounts of Transforming Growth Factor-beta 1 (TGF-beta 1).
  • Elevated serum levels of Interleukin-2 (IL-2), IL-2 receptor (IL-2R), IL-6, and IL-8 were observed.
  • Serum levels of IL-1 beta, IL-4, and tumor necrosis factor-alpha remained within normal ranges.

Implications:

  • This case expands the understanding of LGL leukemia's diverse clinical presentations.
  • The findings highlight the potential role of TGF-beta 1 and specific interleukins in the pathogenesis of this LGL leukemia subtype.
  • Further research may elucidate the mechanisms linking T-cell leukemia, myelofibrosis, and cytokine dysregulation.

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