Jin Xiaoke, Xu Youhai, Zhang Jun, Li Guangxi, Huang Dongping, Yang Yuqiong, He Hesheng
Laboratory of Hematopathology, Department of Hematology, the Affiliated Yijishan Hospital of Wannan Medical College, Wuhu, 241000, China.
Department of Hematology, the Affiliated Yijishan Hospital of Wannan Medical College, Zheshan West Road, Wuhu, 241000, China.
Diagn Pathol. 2017 Jun 17;12(1):46. doi: 10.1186/s13000-017-0636-1.
Aggressive natural killer cell leukemia/lymphoma (ANKL) is a rare and highly aggressive NK cell neoplasm with a short clinical course and poor prognosis and is often misdiagnosed and confused with NK/T cell lymphoma (NKTL), which has a very different prognosis. Here, we present a case with nasal and bone marrow involvement, provide a literature review and make a differential diagnosis.
A 41-year-old male presented nasal congestion pharyngalgia, palatal perforation, high fever and multiorgan dysfunction. Our diagnosis primarily relied on clinical features, the morphology and immunophenotype of the neoplastic cells and imaging studies. Characteristic large granular lymphocytes with azurophilic granules were visible in the bone marrow smears. In addition, the neoplastic cells expressed a typical immunophenotype, and the T cell receptor γ (TCR-γ) gene rearrangement analysis and presence of Epstein-Barr virus (EBV) were negative. The patient's symptoms and signs were temporarily relieved after chemotherapy treatment, but after a short time, he underwent a rapid clinical decline and died 8 weeks later after admission due to multiorgan function failure.
Our case demonstrates that to avoid a misdiagnosis, bone marrow analyses and other examinations should be performed early when a patient initially presents nasal lesions and other systemic symptoms. To the best of our knowledge, this may be the first reported case of ANKL with sternal tenderness.
侵袭性自然杀伤细胞白血病/淋巴瘤（ANKL）是一种罕见且侵袭性很强的NK细胞肿瘤，临床病程短，预后差，常被误诊并与预后差异很大的NK/T细胞淋巴瘤（NKTL）相混淆。在此，我们报告一例伴有鼻腔和骨髓受累的病例，进行文献复习并作出鉴别诊断。
一名41岁男性出现鼻塞、咽痛、腭穿孔、高热和多器官功能障碍。我们的诊断主要依靠临床特征、肿瘤细胞的形态和免疫表型以及影像学检查。骨髓涂片可见特征性的含嗜天青颗粒的大颗粒淋巴细胞。此外，肿瘤细胞表达典型的免疫表型，T细胞受体γ（TCR-γ）基因重排分析及EB病毒（EBV）检测均为阴性。化疗治疗后患者的症状和体征暂时缓解，但不久后病情迅速恶化，入院8周后因多器官功能衰竭死亡。
我们的病例表明，为避免误诊，当患者最初出现鼻腔病变及其他全身症状时，应尽早进行骨髓分析及其他检查。据我们所知，这可能是首例报道的伴有胸骨压痛的ANKL病例。
