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

Autor: Khasawneh A; Department of Pathology, New York University Langone Medical Center, 240 East 38Th Street, 22Nd Floor, New York, NY, USA., McGuinness G; Department of Radiology, New York University Langone Medical Center, New York, NY, USA., Ward N; Department of Pathology, New York University Langone Medical Center, 240 East 38Th Street, 22Nd Floor, New York, NY, USA. Nicholas.Ward@nyulangone.org.
Jazyk: angličtina
Zdroj: Journal of hematopathology [J Hematop] 2024 Mar; Vol. 17 (1), pp. 27-36. Date of Electronic Publication: 2023 Dec 26.
DOI: 10.1007/s12308-023-00572-5
Abstrakt: 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.
(© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
Databáze: MEDLINE
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