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Initial diagnosis of extranodal NK/T-cell lymphoma in pericardial fluid with concomitant hemophagocytic lymphohistiocytosis (HLH)

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Abstract

Extranasal natural killer/T-cell lymphoma arising in the heart is rare and typically presents with non-specific clinical symptoms, necessitating a biopsy for a definitive diagnosis. We report an unusual case of a 48-year-old male who initially presented with chest pain and shortness of breath. Subsequent diagnosis via pericardial fluid analysis, including flow cytometry and immunohistochemical stains, revealed extranasal NK/T-cell lymphoma without sinonasal involvement. The analysis identified neoplastic lymphoid cells expressing CD2, cytoplasmic CD3, Epstein-Barr virus, and CD56 and exhibiting increased Ki-67 staining. Additionally, the patient developed hemophagocytosis lymphocytosis secondary to NK/T cell lymphoma. Treatment included an interleukin-1 receptor antagonist (anakinra), dexamethasone, rituximab, and etoposide. Unfortunately, the patient’s condition rapidly deteriorated, leading to multiorgan failure and eventual demise. Given the rarity of this lymphoma, early diagnosis based on a high suspicion level provides the best chance for improved overall survival.

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Authors and Affiliations

  1. Department of Pathology, New York University Langone Medical Center, 240 East 38Th Street, 22Nd Floor, New York, NY, USA

    Amani Khasawneh & Nicholas Ward

  2. Department of Radiology, New York University Langone Medical Center, New York, NY, USA

    Georgeann McGuinness

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  1. Amani Khasawneh
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  3. Nicholas Ward
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Correspondence to Nicholas Ward.

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Khasawneh, A., McGuinness, G. & Ward, N. Initial diagnosis of extranodal NK/T-cell lymphoma in pericardial fluid with concomitant hemophagocytic lymphohistiocytosis (HLH). J Hematopathol 17, 27–36 (2024). https://doi.org/10.1007/s12308-023-00572-5

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