血管免疫母细胞性T细胞淋巴瘤诱发噬血细胞性淋巴组织细胞增生症和弥散性血管内凝血：一例报告
Angioimmunoblastic T-cell lymphoma induced hemophagocytic lymphohistiocytosis and disseminated intravascular coagulopathy: A case report.
作者信息
Jiang Mei, Wan Jing-Hua, Tu Yi, Shen Yan, Kong Fan-Cong, Zhang Zhang-Lin
机构信息
Clinical Laboratory, The First Affiliated Hospital of Nanchang University, Nanchang 330006, Jiangxi Province, China.
Department of Pathology, The First Affiliated Hospital of Nanchang University, Nanchang 330006, Jiangxi Province, China.
出版信息
World J Clin Cases. 2023 Feb 16;11(5):1086-1093. doi: 10.12998/wjcc.v11.i5.1086.
BACKGROUND
Angioimmunoblastic T-cell lymphoma (AITL) is a subtype of peripheral T-cell lymphoma, with heterogenous clinical manifestations and poor prognosis. Here, we report a case of AITL induced hemophagocytic lymphohistiocytosis (HLH) and disseminated intravascular coagulopathy (DIC).
CASE SUMMARY
An 83-year-old man presented with fever and purpura of both lower limbs for one month. Groin lymph node puncture and flow cytometry indicated a diagnosis of AITL. Bone marrow examination and other laboratory related indexes indicated DIC and HLH. The patient rapidly succumbed to gastrointestinal bleeding and septic shock.
CONCLUSION
This is the first reported case of AITL induced HLH and DIC. AITL is more aggressive in older adults. In addition to male gender, mediastinal lymphadenopathy, anaemia, and sustained high level of neutrophil-to-lymphocyte ratio may indicate a greater risk of death. Early diagnosis, early detection of severe complications, and prompt and effective treatment are vital.
背景
血管免疫母细胞性T细胞淋巴瘤（AITL）是外周T细胞淋巴瘤的一种亚型，临床表现多样，预后较差。在此，我们报告一例AITL诱发噬血细胞性淋巴组织细胞增生症（HLH）和弥散性血管内凝血（DIC）的病例。
病例摘要
一名83岁男性，出现发热及双下肢紫癜1个月。腹股沟淋巴结穿刺及流式细胞术检查提示诊断为AITL。骨髓检查及其他实验室相关指标提示存在DIC和HLH。患者迅速死于消化道出血和感染性休克。
结论
这是首例报道的AITL诱发HLH和DIC的病例。AITL在老年人中侵袭性更强。除男性外，纵隔淋巴结肿大、贫血以及持续高水平的中性粒细胞与淋巴细胞比值可能提示更高的死亡风险。早期诊断、早期发现严重并发症并及时有效治疗至关重要。
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