title: Aggressive natural killer-cell leukemia with jaundice and spontaneous splenic rupture: a case report and review of the literature.
abstract: Aggressive natural killer cell leukemia/lymphoma (ANKL) is a rare aggressive form of NK-cell neoplasm. We report an uncommon case of 36-year-old male who showed jaundice and spontaneous splenic rupture. The diagnosis was established by the biopsy of liver and spleen. The monomorphous medium-size neoplastic cells infiltrated into portal areas and sinus of liver as well as the cords and sinus of the spleen. Necrosis, mitotic figures and significant apoptosis could be seen easily. These neoplastic cells demonstrated a typical immunophenotype of CD3ε+, CD56+, CD16+, Granzyme B+, TIA-1+. T-cell receptor γ (TCR-γ) gene rearrangement analysis showed germline configuration and the result of in situ hybridization for Epstein-Barr virus-encoded RNA (EBER-ISH) was positive. The patient has undergone an aggressive clinical course and died of multi-organ function failure 14 days later after admission. To the best of our knowledge, this is the first case of ANKL with spontaneous splenic rupture, and we should pay more attention to recognize it.
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authors.affiliation: Department of Pathology, West China Hospital of Sichuan University, Chengdu 610041, China.
authors.forename: Li-min
authors.initials: LM
authors.lastname: Gao
authors.forename: Wei-ping
authors.initials: WP
authors.lastname: Liu
authors.forename: Qun-pei
authors.initials: QP
authors.lastname: Yang
authors.forename: Hui-fang
authors.initials: HF
authors.lastname: Li
authors.forename: Jun-jie
authors.initials: JJ
authors.lastname: Chen
authors.forename: Yuan
authors.initials: Y
authors.lastname: Tang
authors.forename: Yan
authors.initials: Y
authors.lastname: Zou
authors.forename: Dian-Ying
authors.initials: DY
authors.lastname: Liao
authors.forename: Yan-mei
authors.initials: YM
authors.lastname: Liu
authors.forename: Sha
authors.initials: S
authors.lastname: Zhao
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis / Q000235:genetics; D001706:Biopsy; D017809:Fatal Outcome; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D019675:Genes, T-Cell Receptor gamma; D004854:Herpesvirus 4, Human / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D007565:Jaundice / Q000209:etiology* / Q000276:immunology / Q000473:pathology / Q000628:therapy; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000150:complications* / Q000235:genetics / Q000276:immunology / Q000473:pathology / Q000628:therapy / Q000821:virology; D008099:Liver / Q000473:pathology; D008297:Male; D055611:Natural Killer T-Cells / Q000276:immunology / Q000473:pathology*; D012367:RNA, Viral / Q000032:analysis; D012422:Rupture, Spontaneous; D013154:Spleen / Q000473:pathology; D013161:Splenic Rupture / Q000209:etiology* / Q000276:immunology / Q000473:pathology / Q000628:therapy; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor; D012367:RNA, Viral