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Open Access Highly Accessed Case Report

Aggressive natural killer-cell leukemia with jaundice and spontaneous splenic rupture: a case report and review of the literature

Li-min Gao1, Wei-ping Liu1*, Qun-pei Yang1, Hui-fang Li2, Jun-jie Chen3, Yuan Tang1, Yan Zou1, Dian-Ying Liao1, Yan-mei Liu1 and Sha Zhao1*

Author affiliations

1 Department of Pathology, West China Hospital of Sichuan University, Chengdu 610041, China

2 Laboratory of Stem Cell Biology, State Key Laboratory of Biotherapy, West China Hospital of Sichuan University, Chengdu 610041, China

3 Department of Burns and Plastic Surgery, West China Hospital of Sichuan University, Chengdu 610041, China

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Citation and License

Diagnostic Pathology 2013, 8:43  doi:10.1186/1746-1596-8-43

Published: 11 March 2013

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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Keywords:
Aggressive natural killer-cell leukemia; Jaundice; Spontaneous splenic rupture