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26-12-2023 | Lymphoma | Case Report

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

Authors: Amani Khasawneh, Georgeann McGuinness, Nicholas Ward

Published in: Journal of Hematopathology | Issue 1/2024

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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Title: Initial diagnosis of extranodal NK/T-cell lymphoma in pericardial fluid with concomitant hemophagocytic lymphohistiocytosis (HLH)
Authors: Amani Khasawneh
Georgeann McGuinness
Nicholas Ward
Publication date: 26-12-2023
Publisher: Springer Berlin Heidelberg
Keywords: Lymphoma
Epstein-Barr Virus
Hemophagocytic Lymphohistiocytosis
Hemophagocytic Lymphohistiocytosis
Published in: Journal of Hematopathology / Issue 1/2024
Print ISSN: 1868-9256
Electronic ISSN: 1865-5785
DOI: https://doi.org/10.1007/s12308-023-00572-5

ERS Congress 2024

Springer Medicine

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

Abstract

ERS Congress 2024

Springer Medicine

Abstract

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