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
in 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.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Pericardial Effusion
/
Lymphoma, T-Cell, Peripheral
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Epstein-Barr Virus Infections
/
Lymphohistiocytosis, Hemophagocytic
/
Lymphoma, Extranodal NK-T-Cell
Limits:
Humans
/
Male
/
Middle aged
Language:
En
Journal:
J Hematop
/
J. hematop. (Internet)
/
Journal of hematopathology (Internet)
Year:
2024
Document type:
Article
Affiliation country:
Estados Unidos
Country of publication:
Alemania