A Case Report of Primary Nasal Natural Killer (NK)/T-Cell Lymphoma in an African American Patient Presenting with Hemophagocytic Syndrome

Abstract

Patient: Male, 55Final Diagnosis: Primary NK-T cell lymphoma • nasal typeSymptoms: Fever • nasal bleeding • nasal mass • weight lossMedication: —Clinical Procedure: Chemotherapy×2 cycles • radiation therapSpecialty: OncologyObjective:Rare diseaseBackground: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.

Extreme hyperferritinemia without associated HLH in a patient with T‐cell lymphoma

“…Most reported cases of PTCL associated with HLH and hyperferritinemia in the literature involve extranodal nature killer/T-cell lymphoma, nasal subtype with very few investigating the association of marked hyperferritinemia, HLH, and ALCL. 14,15,17 However, of those reported cases of ALCL with associated hyperferritinemia and/or HLH, poorer outcomes and decreased overall survival have been demonstrated. 19,20 This observation highlights the importance of arriving at a timely and accurate diagnosis in cases of hyperferritinemia resulting from T-cell lymphoma (with or without HLH), as diagnostic delay may impact progression of disease and prognosis.…”

“…Most reported cases of PTCL associated with HLH and hyperferritinemia in the literature involve extranodal nature killer/T‐cell lymphoma, nasal subtype with very few investigating the association of marked hyperferritinemia, HLH, and ALCL 14,15,17 . However, of those reported cases of ALCL with associated hyperferritinemia and/or HLH, poorer outcomes and decreased overall survival have been demonstrated 19,20 .…”

“…In the existing literature, there are several case reports of marked hyperferritinemia in patients diagnosed with T‐cell lymphoma. However, reported cases have all been found to have associated secondary HLH 13–20 …”

Inert CD30-positive extranodal NK/T cell lymphoma with large cell transformation: a case report

“…Serious patients accompanied by Haemophilus syndrome severe and clinical manifestations such as high fever, hepatosplenomegaly, hemocytopenias. Under a microscope, the mass showed extensive necrosis and inflammatory exudation, Tumor cells were medium to large in size with irregular nuclei; some can see anaplastic cells, showed round or ovoid, and a moderate amount of cytoplasm which characteristically showed peculiar coarse eosinophilic granules, and eggplant-like kidney-like irregular nuclei, unobvious nucleoli (2). The lesion was mainly infiltrated by small-to middle-size atypical lymphoid cell, histiocytes and eosinophils.…”

A case of primary cutaneous extranodal NK/T‐cell lymphoma in an African male

“…It is often misdiagnosed due to its similar presentation to other cutaneous lymphomas. PC‐ENKTL is most common in East Asians and Latin Americans but is rarely reported in those of African descent. We report the case of an African male with a bullous presentation of PC‐ENKTL.…”