Hust Michael A, Blechacz Boris R A, Bonilla Diana L, Daver Naval, Rojas-Hernandez Cristhiam M
Department of Internal Medicine, The University of Texas Health Science Center, Houston, TX, USA.
Department of Gastroenterology, Hepatology and Nutrition, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
J Med Case Rep. 2017 Jun 27;11(1):172. doi: 10.1186/s13256-017-1344-x.
Adult hemophagocytic lymphohistiocytosis is a secondary immunopathologic phenomenon, mainly secondary to malignancy, infection, or autoimmune disorders. The performance of diagnostic criteria, studied in the pediatric population, is yet to be validated in the adult population. Some of the criteria include cytopenias and organomegaly that are inherent features to malignant processes, thus making the diagnosis of hemophagocytic lymphohistiocytosis a challenge in patients with cancer.
We describe the case of a 54-year-old white man with history of metastatic maxillary sinus adenoid cystic carcinoma who had severe liver injury and cytopenias with progressive clinical deterioration. We performed an evaluation, by flow cytometry, of the expression of surface markers in his natural killer cells that revealed remarkable abnormalities. His syndrome eventually fulfilled criteria for hemophagocytic lymphohistiocytosis and he received therapy with steroids with interval clinical improvement. Unfortunately, he refused further cytotoxic treatment and died 2 weeks later.
The conventional criteria for the diagnosis of hemophagocytic lymphohistiocytosis are suboptimal for adult patients with cancer resulting in delays in diagnosis and timely initiation of treatment. The diagnostic criteria have to be re-evaluated in patients with cancer; novel, easily available, and accurate diagnostic methods are needed.
成人噬血细胞性淋巴组织细胞增生症是一种继发性免疫病理现象，主要继发于恶性肿瘤、感染或自身免疫性疾病。在儿科人群中研究的诊断标准在成人人群中尚未得到验证。其中一些标准包括血细胞减少和器官肿大，这些是恶性疾病的固有特征，因此对于癌症患者而言，噬血细胞性淋巴组织细胞增生症的诊断具有挑战性。
我们描述了一名54岁白人男性的病例，他有转移性上颌窦腺样囊性癌病史，出现严重肝损伤和血细胞减少，临床症状逐渐恶化。我们通过流式细胞术评估了他自然杀伤细胞表面标志物的表达，结果显示明显异常。他的综合征最终符合噬血细胞性淋巴组织细胞增生症的标准，接受了类固醇治疗，临床症状有所改善。不幸的是，他拒绝进一步的细胞毒性治疗，两周后死亡。
噬血细胞性淋巴组织细胞增生症的传统诊断标准对成年癌症患者并不理想，导致诊断延迟和治疗的及时启动受阻。对于癌症患者必须重新评估诊断标准；需要新的、易于获得且准确的诊断方法。
