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Report of an HIV and HHV-8 negative case of primary effusion lymphoma with idiopathic T4 lymphocytopenia

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Abstract

Although primary effusion lymphoma (PEL) is usually associated with human herpes virus-8/Kaposi sarcoma herpes virus (HHV-8/KSHV) and human immunodeficiency virus (HIV), there are several reports of HHV-8/KSHV and HIV negative cases, mainly in the setting of immunodeficiency. Here, we report the second case of PEL associated with idiopathic T4 lymphocytopenia (ICL), which was HHV-8/KSHV negative, HIV negative and Epstein-Barr virus positive, while no other causative agents for immunodeficiency were documented. Flow cytometry revealed a hyperdiploid and highly mitotic large B-cell population, CD30, EMA, CD66, CD38 and CD71 positive. The malignant lymphoma cells showed atypia with prominent nuclei and basophilic vacuolated cytoplasm, while cytogenetic analysis with fluorescent in situ hybridization showed trisomy 18. The patient was administered R-COP chemotherapy, but no remission was achieved, up to 3 months from diagnosis.

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Authors and Affiliations

  1. Flow Cytometry Laboratory, Department of Immunology, Athens Regional General Hospital “G. Gennimatas”, Mesogion Avenue 154, 11527, Athens, Greece

    Nikolaos J. Tsagarakis, Nektaria Kentrou, Konstantinos A. Papadimitriou, Dimitra Skoumi & Georgios Paterakis

  2. Department of Clinical Hematology, Athens Regional General Hospital “G. Gennimatas”, Athens, Greece

    Aspasia Argyrou & Georgios Gortzolidis

  3. Department of Laboratory Hematology, Athens Regional General Hospital “G. Gennimatas”, Athens, Greece

    Stefanos I. Papadhimitriou

  4. Department of Microbiology, Athens Regional General Hospital “G. Gennimatas”, Athens, Greece

    Konstantina Tzanetou

  5. Department of Pathology, Athens Regional General Hospital “G. Gennimatas”, Athens, Greece

    Georgios Kakiopoulos

Authors
  1. Nikolaos J. Tsagarakis
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  2. Aspasia Argyrou
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  3. Georgios Gortzolidis
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  4. Nektaria Kentrou
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  5. Stefanos I. Papadhimitriou
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  8. Konstantinos A. Papadimitriou
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  9. Dimitra Skoumi
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  10. Georgios Paterakis
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Correspondence to Nikolaos J. Tsagarakis.

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Tsagarakis, N.J., Argyrou, A., Gortzolidis, G. et al. Report of an HIV and HHV-8 negative case of primary effusion lymphoma with idiopathic T4 lymphocytopenia. Int J Hematol 90, 94–98 (2009). https://doi.org/10.1007/s12185-009-0343-0

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  • DOI: https://doi.org/10.1007/s12185-009-0343-0

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