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T-cell Prolymphocytic Leukemia, Cerebriform Variant.

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  • 1Internal Medicine, Unidade Local de Saúde do Alto Minho (ULSAM), Viana do Castelo, PRT.

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Summary

T-cell prolymphocytic leukemia (T-PLL), a rare aggressive cancer, rapidly progressed in a 70-year-old man. The case highlights a specific variant with cerebriform nuclei, leading to fatal tumor lysis syndrome.

Keywords:
adenopathycerebriformlymphocytosissplenomegalyt-cell leukemia

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Area of Science:

  • Hematology
  • Oncology

Background:

  • T-cell prolymphocytic leukemia (T-PLL) is an aggressive lymphoid malignancy.
  • It is characterized by rapid proliferation of mature T-lymphocytes.

Observation:

  • A 70-year-old male presented with fatigue, decreased urine output, and edema.
  • Clinical findings included diminished breath sounds, peripheral edema, anemia, acute kidney injury, and pleural effusion.
  • Subsequent development of thrombocytopenia, worsening lymphocytosis, adenopathy, and splenomegaly were noted.

Findings:

  • Immunophenotypic analysis confirmed T-PLL.
  • Negative HTLV-1 serology ruled out adult T-cell leukemia.
  • Peripheral blood smear revealed lymphocytes with cerebriform nuclei, indicating a specific T-PLL variant.

Implications:

  • This case underscores the aggressive nature of T-PLL.
  • The identification of the cerebriform nucleus variant highlights diagnostic nuances.
  • Rapid progression and tumor lysis syndrome emphasize the need for prompt management strategies.