A young woman with persistent sore throat, <scp>Epstein–Barr</scp> virus, lymphadenopathy, and aberrant <scp>CD4</scp> + <scp>CD7</scp>‐ T‐cells

Goubran, Mariam; McGinnis, Eric; Stubbins, Ryan J.; Nicolson, Hamish; Pourshahnazari, Persia; Belga, Sara; Merkeley, Hayley; Nevill, Thomas J.; Chen, Luke Y C

doi:10.1002/ajh.26838

American J Hematol

2023

DOI: 10.1002/ajh.26838

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A young woman with persistent sore throat, Epstein–Barr virus, lymphadenopathy, and aberrant CD4 + CD7‐ T‐cells

Mariam Goubran

Eric McGinnis

Ryan J. Stubbins

et al.

Abstract: A young woman with persistent EBV viremia and lymphocytosis had an abnormal CD4‐ T cell population with aberrant loss of CD7. She had a diagnosis of chronic active EBV (CAEBV), a lymphoproliferative disorder for which she ultimately required allogeneic hematopoietic stem cell transplantation.

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2024

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Case report: Aggressive natural killer cell leukemia and refractory hemophagocytic lymphohistiocytosis in an adolescent

Spaner,

Durkee-Shock,

Weng

et al. 2024

Front. Hematol.

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Aggressive natural killer cell leukemia (ANKL) is a rare, aggressive hematologic malignancy which often presents as fulminant Epstein-Barr virus (EBV)- driven hemophagocytic lymphohistiocytosis (HLH). ANKL lacks a distinct immunologic and morphologic signature, making early diagnosis particularly challenging. Here we present a case of ANKL in a patient presenting with EBV-HLH. After poor treatment response to the HLH-2004 protocol (etoposide and dexamethasone), bone marrow biopsy demonstrated an atypical CD3-/CD56+ natural killer (NK) cell population with diminished CD7 expression consistent with EBV+ ANKL. Asparaginase-based chemotherapy was initiated but his disease progressed and he died from multiorgan failure. This case highlights the diagnostic challenges of ANKL given the lack of standardized diagnostic criteria, the importance of considering T/NK cell malignancies in the differential diagnosis of EBV-HLH, and adds to the literature on this rare disease.

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Case report: Aggressive natural killer cell leukemia and refractory hemophagocytic lymphohistiocytosis in an adolescent

Spaner,

Durkee-Shock,

Weng

et al. 2024

Front. Hematol.

View full text Add to dashboard Cite

show abstract

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A young woman with persistent sore throat, Epstein–Barr virus, lymphadenopathy, and aberrant CD4 + CD7‐ T‐cells

Cited by 1 publication

References 35 publications

Case report: Aggressive natural killer cell leukemia and refractory hemophagocytic lymphohistiocytosis in an adolescent

Case report: Aggressive natural killer cell leukemia and refractory hemophagocytic lymphohistiocytosis in an adolescent

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