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T-Cell Large Granular Lymphocytic Leukemia - Case Reports

Sandra Kojić Katović1, Ankica Vasilj1, Goran Rinčić2

  • 1Department of Cytology, Sestre milosrdnice University Hospital Centre, Zagreb, Croatia.

Acta Clinica Croatica
|November 16, 2018
PubMed

Insights

T-cell large granular lymphocytic leukemia (T-LGLL) is a rare clonal lymphoid disorder. This case highlights diagnosis and successful treatment of T-LGLL presenting with abdominal pain and splenomegaly.

Area of Science:

  • Hematology
  • Oncology
  • Immunology

Background:

  • T-cell large granular lymphocytic leukemia (T-LGLL) is a clonal proliferation of large granular lymphocytes, often underdiagnosed.
  • Diagnosis relies on characteristic lymphoid cell morphology and abnormal immunophenotype via flow cytometry.
  • Its indolent nature frequently makes observation a suitable initial management strategy.

Observation:

  • A 53-year-old male presented with abdominal pain, mild microcytic anemia, and elevated lactate dehydrogenase.
  • Abdominal ultrasound revealed significant splenomegaly (16 cm) without lymphadenopathy.
  • Bone marrow examination showed hypocellularity with 50% atypical lymphoid cells and peripheral blood revealed 81% atypical granular lymphocytes.

Findings:

  • Bone marrow biopsy confirmed nodular and interstitial proliferation of T-lymphocytes (CD2+, CD3+, CD5+, CD8+, granzyme+, TIA+), consistent with T-LGLL.
  • Immunophenotyping excluded other lymphoid disorders.
  • The patient's presentation included splenomegaly and elevated LDH.

Implications:

  • This case underscores the importance of considering T-LGLL in patients with unexplained cytopenias and splenomegaly.
  • Treatment with cyclosporine and corticosteroids led to regression of splenomegaly and normalization of LDH.
  • Early diagnosis and appropriate management can improve outcomes for T-LGLL patients.

Keywords:
AnemiaBone Marrow, Aspiration and BiopsyCase reportsCell ProliferationFlow CytometryImmunophenotypingLeukemia, Large Granular LymphocyticLymphocytesSplenomegaly

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