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Case 105 - Discussion

Hematopathology Case

Diagnosis: Hepatosplenic Gamma-Delta T-Cell Lymphoma

Flow cytometry showed a population of medium to large cells expressing CD3 and gamma-delta T-cell receptor. The cells were negative for EBV.

 

Hepatosplenic  T-Cell Lymphoma (HSTL)

CLINICAL FEATURES

  • Very rare. Young male, median age 35 years, 86% males.

  • ~20% arise in immune suppression, particularly iatrogenic causes.

  • Hepatosplenomegaly, consistently bone marrow involvement, no lymphadenopathy.

  • Moderate anemia, marked thrombocytopenia. Peripheral blood involvement uncommon at presentation, may occur later in the clinical course.

GROSS FINDINGS

  • The spleen is enlarged with diffuse involvement in the red pulp, without any gross lesions. Diffuse hepatomegaly is also present.

MICROSCOPIC FINDINGS

  • Tumor cells involve the splenic red pulp, hepatic sinusoids and bone marrow sinuses.
  • Monotonous medium sized tumor cells with scant to moderate clear cytoplasm, round/oval nuclei, slightly dispersed chromatin, small inconspicuous nucleoli.
  • Bone marrow aspirate smears contain malignant cells resembling blasts, occasionally with fine cytoplasmic granules.

IMMUNOPHENTYPE

  • CD2+, CD3+, CD7+, CD56+, CD57+, TIA-1+, CD40L+
  • CD4-, CD5-, variable CD8

CYTOGENETIC STUDIES

  • Positive for gamma-delta TCR in most cases, a small proportion of cases positive for alpha-beta TCR.
  • Isochromosome 7 in most of cases

TREATMENT AND PROGNOSIS

  • Aggressive, mostly die with 2 years.

REFERENCES: WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, Fourth Edition. 2008.