title: A Case Report of Primary Nasal Natural Killer (NK)/T-Cell Lymphoma in an African American Patient Presenting with Hemophagocytic Syndrome.
abstract: BACKGROUND Extranodal natural killer/T-cell lymphoma, nasal type (ENKTCL) is generally an aggressive and rare non-Hodgkin lymphoma. It is most common in East Asians, Native Americans, and South Americans, but is rarely reported in blacks. CASE REPORT A 55-year-old African American male born in Grenada presented with a left nostril mass with facial swelling and biopsy subsequently confirmed a diagnosis of extranodal NK/T-cell lymphoma, nasal type (ENKTCL). Immunochemistry was positive for CD2, cytoplasmic CD3, CD7, CD 43, CD 56, granzyme B, and TIA-1. In situ hybridization was positive for Epstein-Barr virus encoded ribonucleic acid (EBERs). Bone marrow aspiration did not show lymphoma involvement. The patient had progressive neutropenia upon presentation, with further investigations showing hepatomegaly, hyperferritinemia, and hemophagocytosis in the bone marrow. We reached a diagnosis of hemophagocytic syndrome. He was treated with a high-dose combination chemotherapy and radiation therapy; the neutropenia improved significantly with steroids as treatment for immune activation in the setting of hemophagocytic syndrome. CONCLUSIONS To the best of our knowledge, this is the only second report of extranodal NK/T-cell lymphoma, nasal type in a black patient, and it raises the awareness of early recognition of rare manifestations of NK/T-cell lymphoma such as hemophagocytic syndrome.
authors.affiliation: Department of Medicine, Brookdale University Hospital and Medical Center, Brooklyn, NY, USA.
authors.forename: Bowei
authors.initials: B
authors.lastname: Tan
authors.affiliation: Department of Medicine, Brookdale University Hospital and Medical Center, Brooklyn, NY, USA.
authors.forename: Cherif
authors.initials: C
authors.lastname: Abdelmalek
authors.affiliation: Department of Medicine, Brookdale University Hospital and Medical Center, Brooklyn, NY, USA.
authors.forename: James E
authors.initials: JE
authors.lastname: O'Donnell
authors.affiliation: Department of Medicine, Brookdale University Hospital and Medical Center, Brooklyn, NY, USA.
authors.forename: Thomas
authors.initials: T
authors.lastname: Toltaku
authors.affiliation: Department of Medicine, Brookdale University Hospital and Medical Center, Brooklyn, NY, USA.
authors.forename: Rashid
authors.initials: R
authors.lastname: Chaudhry
authors.affiliation: Department of Medicine, Brookdale University Hospital and Medical Center, Brooklyn, NY, USA.
authors.forename: Jen C
authors.initials: JC
authors.lastname: Wang
authors.affiliation: Department of Medicine, Brookdale University Hospital and Medical Center, Brooklyn, NY, USA.
authors.forename: Vladimir
authors.initials: V
authors.lastname: Gotlieb
mesh_terms: D001741:Black or African American; D014408:Biomarkers, Tumor; D059248:Chemoradiotherapy; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis* / Q000208:ethnology / Q000628:therapy; D054391:Lymphoma, Extranodal NK-T-Cell / Q000175:diagnosis* / Q000208:ethnology / Q000378:metabolism / Q000628:therapy; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor