Initial diagnosis of extranodal NK/T-cell lymphoma in pericardial fluid with concomitant hemophagocytic lymphohistiocytosis (HLH).
J Hematop
; 17(1): 27-36, 2024 Mar.
Article
en En
| MEDLINE
| ID: mdl-38376724
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.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Derrame Pericárdico
/
Linfoma de Células T Periférico
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Infecciones por Virus de Epstein-Barr
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Linfohistiocitosis Hemofagocítica
/
Linfoma Extranodal de Células NK-T
Límite:
Humans
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Male
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Middle aged
Idioma:
En
Revista:
J Hematop
Año:
2024
Tipo del documento:
Article
País de afiliación:
Estados Unidos