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Occult hepatosplenic T-γδ lymphoma

Value of genotypic analysis in the differential diagnosis

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Abstract

We report on a patient with a rare hepatosplenic γδ T-cell lymphoma (γδ TCL) presenting clinically with B-symptoms, hepatosplenomegaly and pancytopenia. During the initial stage of the disease the sparse malignant cells could not be detected histologically. Furthermore, their identification was obscured by massive macrophage proliferation with haemophagocytosis in the spleen. Diagnosis was established by detection of a clonal T-cell receptor (TcR) rearrangement and, retrospectively, by demonstration of rare cells expressing an aberrant T-cell phenotype. The findings in this patient emphasize that minimal neoplastic T-cell infiltrates can lead to severe clinical symptoms. Initial biopsy findings may be misinterpreted as benign. γδ TCL may elaborate lymphokines that suppress haematopoiesis, leading to pancytopenia and macrophage proliferation.

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Correspondence to C. C. Dommann-Scherrer.

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Dommann-Scherrer, C.C., Zimmermann, D.R., Odermatt, B.F. et al. Occult hepatosplenic T-γδ lymphoma. Vichows Archiv A Pathol Anat 426, 629–634 (1995). https://doi.org/10.1007/BF00192119

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Key words

  • T-cell lymphoma
  • γδ T-cell receptor
  • Spleen
  • Liver
  • Haemophagocytosis