title: Aggressive natural killer-cell neoplasm presenting in the marrow: a report of two cases including one with gains of chromosomes 4q and 9p.
abstract: Aggressive nature killer (NK)-cell neoplasm includes aggressive NK-cell leukemia (ANKL) and extranodal NK/T-cell lymphoma (ENKTL), nasal type. ANKL is rare and is characterized by a systemic neoplastic proliferation of NK-cells, usually with a leukemic presentation. ENKTL is a predominantly extranodal lymphoma, occurring mainly in the upper aerodigestive tract. Both are aggressive neoplasms strongly associated with Epstein-Barr virus (EBV). Here we report two patients with aggressive NK-cells neoplasms localized in the bone marrow (BM) who presented as prolonged fever, anemia, and thrombocytopenia. Both were treated initially as infectious disease. Imaging studies revealed splenomegaly without any nodular lesion or lymphadenopathy. BM examination revealed extensive involvement by EBV-positive NK-cells in both cases. Staging workup including nasal examination/biopsy was negative. Both patients passed away in a month. One case showed gains of chromosomes 4q and 9p by array comparative genomic hybridization. Both tumors were diagnostically challenging due to the unusual clinical presentation and absence of leukemic change, tumor mass or lymphadenopathy. Our cases demonstrate that lymphoma should be considered in patients with fever of unknown origin and bone marrow aspiration/biopsy should be performed as early diagnosis and novel therapeutic regimens may benefit these patients.
authors.affiliation: Department of Anatomic Pathology, Far Eastern Memorial Hospital, New Taipei City, Taiwan.
authors.forename: Jie-Yang
authors.initials: JY
authors.lastname: Jhuang
authors.affiliation: Department of Pathology, University of Cambridge, Cambridge, UK.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Clipson
authors.affiliation: Department of Pathology, Chi-Mei Medical Centre, 901 Chung-Hwa Road, Yung-Kang District, Tainan, Taiwan.
authors.forename: Yen-Chuan
authors.initials: YC
authors.lastname: Hsieh
authors.affiliation: Departments of Critical Care Medicine and Pathology, Chi-Mei Medical Center, Tainan, Taiwan.
authors.forename: Chun-Chieh
authors.initials: CC
authors.lastname: Yang
authors.affiliation: Department of Pathology, Chi-Mei Medical Centre, 901 Chung-Hwa Road, Yung-Kang District, Tainan, Taiwan.|Department of Nursing, National Tainan Institute of Nursing, Tainan, Taiwan.
authors.forename: Sheng-Tsung
authors.initials: ST
authors.lastname: Chang
authors.affiliation: Department of Pathology, University of Cambridge, Cambridge, UK.
authors.forename: Ming-Qing
authors.initials: MQ
authors.lastname: Du
authors.affiliation: Department of Pathology, Chi-Mei Medical Centre, 901 Chung-Hwa Road, Yung-Kang District, Tainan, Taiwan. cmh5301@mail.chimei.org.tw.|Department of Pathology, College of Medicine, National Taiwan University and Taipei Medical University, Taipei, Taiwan. cmh5301@mail.chimei.org.tw.
authors.forename: Shih-Sung
authors.initials: SS
authors.lastname: Chuang
mesh_terms: D014408:Biomarkers, Tumor / Q000032:analysis / Q000235:genetics*; D001706:Biopsy; D001856:Bone Marrow Examination; D019046:Bone Marrow Neoplasms / Q000235:genetics* / Q000276:immunology / Q000473:pathology / Q000628:therapy / Q000821:virology; D002869:Chromosome Aberrations; D002894:Chromosomes, Human, Pair 4; D002899:Chromosomes, Human, Pair 9; D055028:Comparative Genomic Hybridization; D003937:Diagnosis, Differential; D003951:Diagnostic Errors; D017809:Fatal Outcome; D005260:Female; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D006801:Humans; D054391:Lymphoma, Extranodal NK-T-Cell / Q000235:genetics* / Q000276:immunology / Q000473:pathology / Q000628:therapy / Q000821:virology; D008297:Male; D008875:Middle Aged; D055611:Natural Killer T-Cells / Q000276:immunology / Q000473:pathology* / Q000821:virology; D011237:Predictive Value of Tests; D013997:Time Factors
chemical_list: D014408:Biomarkers, Tumor