EB病毒相关嗜血细胞综合征1例报道并文献复习
EB Virus-Associated Hemophilic Syndrome: A Case Report and Literature Review
摘要: 目的:探讨成人EB病毒(EBV)相关嗜血细胞综合征(HPS)的临床症状体征和影像学表现,提高临床诊断准确率,减少漏诊和误诊。方法:回顾性分析2021年11月西安医学院第一附属医院呼吸与危重症医学科收治的1例成人EBV相关HPS患者的资料,分析诊疗过程。结果:患者主要临床表现为发热、血小板减少和脾肿大、胆汁淤积、脂肪肝,血NGS对EB病毒DNA检测阳性,结合病史查体及辅助检查结果明确诊断为EB病毒相关嗜血细胞综合征。结论:成人EBV相关HPS临床表现多变,缺乏特异性,血NGS检测和腹部CT可协助明确诊断,提高了临床医师对该病的诊出率,减少误诊漏诊的发生。
Abstract: Objective: To explore the clinical symptoms, signs and imaging findings of adult EB virus (EBV)- as-sociated hemophilic syndrome (HPS), so as to improve the accuracy of clinical diagnosis and reduce missed diagnosis and misdiagnosis. Method: The data of an adult patient with EBV-related HPS treated in the Department of Respiratory and Critical Care in The First Affiliated Hospital of Xi’an Medical College in November 2021 were analyzed retrospectively. Results: The main clinical mani-festations of the patients were fever, thrombocytopenia and splenomegaly, cholestasis and fatty liver. Blood NGS was positive for EB virus DNA. Combined with medical history and auxiliary exam-ination, the patient was diagnosed as EB virus-associated hemophilia syndrome. Conclusion: The clinical manifestations of adult EBV-related HPS are changeable and lack of specificity. The detec-tion of blood NGS and abdominal CT can help to make a clear diagnosis, improve the diagnosis rate of clinicians and reduce the occurrence of misdiagnosis and missed diagnosis.
文章引用:翁俊娜, 高飞. EB病毒相关嗜血细胞综合征1例报道并文献复习[J]. 临床医学进展, 2024, 14(2): 2953-2958. https://doi.org/10.12677/ACM.2024.142418

参考文献

[1] Liu, C., Li, M., Wu, X., et al. (2018) Type 2 Familial Hemophagocytic Lymphohistiocytosis in Half Brothers: A Case Report. Medicine, 97, e11577. [Google Scholar] [CrossRef
[2] Imashuku, S., Morimoto, A. and Ishii, E. (2021) Virus-Triggered Secondary Hemophagocytic Lymphohistiocytosis. Acta Paediatrica, 110, 2729-2736. [Google Scholar] [CrossRef] [PubMed]
[3] 谢正德, 申昆玲. 重视儿童非肿瘤性EB病毒感染疾病的研究[J]. 首都医科大学学报, 2010, 31(2): 213-216.
[4] Hines, M.R., Von Bahr Greenwood, T., Beutel, G., et al. (2022) Consensus-Based Guidelines for the Recognition, Diagnosis, and Management of Hemophagocytic Lymphohistiocytosis in Critically Ill Children and Adults. Critical Care Medicine, 50, 860-872. [Google Scholar] [CrossRef
[5] Otrock, Z.K. and Eby, C.S. (2015) Clinical Characteristics, Prognostic Factors, and Outcomes of Adult Patients with Hemophagocytic Lymphohistiocytosis. American Journal of Hematology, 90, 220-224. [Google Scholar] [CrossRef] [PubMed]
[6] Henter, J.I., Samuelsson-Horne, A., Aricò, M., et al. (2002) Treatment of Hemophagocytic Lymphohistiocytosis with HLH-94 Immunochemotherapy and Bone Marrow Transplantation. Blood, 100, 2367-2373. [Google Scholar] [CrossRef] [PubMed]
[7] 丁秀云. 小儿噬血细胞综合征误诊5例分析[J]. 中国误诊学杂志, 2006, 6(10): 1911-1912.
[8] 中国医师协会血液科医师分会, 中华医学会儿科学分会血液学组, 噬血细胞综合征中国专家联盟. 中国噬血细胞综合征诊断与治疗指南(2022年版) [J]. 中华医学杂志, 2022, 102(20): 1492-1499.
[9] Ishii, E., Ohga, S., Imashuku, S., et al. (2007) Nationwide Survey of Hemophagocytic Lymphohistio-cytosis in Japan. International Journal of Hematology, 86, 58-65. [Google Scholar] [CrossRef
[10] Park, H.S., Lee, J.H., Lee, J.H., et al. (2019) Fludarabine/Melphalan 100 mg/m2 Conditioning Therapy Followed by Allogeneic Hematopoietic Cell Transplantation for Adult Patients with Secondary Hemophagocytic Lymphohistiocytosis. Trans-plantation and Cellular Therapy, 25, 1116-1121. [Google Scholar] [CrossRef] [PubMed]
[11] Taylor, G.S., Long, H.M., Brooks, J.M., et al. (2015) The Immunology of Epstein-Barr Virus-Induced Disease. Annual Review of Immunology, 33, 787-821. [Google Scholar] [CrossRef] [PubMed]
[12] Lindemann, T.L. and Greene, J.S. (2005) Persistent Cervical Lymphadenopathy in an Adolescent with Epstein-Barr Induced Hemophagocytic Syndrome: Manifestations of a Rare But Often Fatal Disease. International Journal of Pediatric Otorhinolaryngology, 69, 1011-1014. [Google Scholar] [CrossRef] [PubMed]
[13] Fujiwara, S., Kimura, H., Imadome, K., et al. (2014) Current Research on Chronic Active Epstein-Barr Virus Infection in Japan. Pediatrics International, 56, 159-166. [Google Scholar] [CrossRef] [PubMed]
[14] 杨骏, 张永红, 谢正德, 等. 儿童EB病毒相关噬血淋巴组织细胞增生症26例[J]. 中国小儿血液与肿瘤杂志, 2007, 12(4): 157-160.
[15] Henter, J.I., Horne, A., Aricó, M., et al. (2007) HLH-2004: Diagnostic and Therapeutic Guidelines for Hemophagocytic Lymphohistiocytosis. Pediatric Blood & Cancer, 48, 124-131. [Google Scholar] [CrossRef] [PubMed]
[16] Wang, Y., Huang, W., Hu, L., et al. (2015) Multicenter Study of Combination DEP Regimen as a Salvage Therapy for Adult Refractory Hemophagocytic Lymphohistiocytosis. Blood, 126, 2186-2192. [Google Scholar] [CrossRef] [PubMed]
[17] Parikh, S.A., Kapoor, P., Letendre, L., et al. (2014) Prognostic Factors and Outcomes of Adults with Hemophagocytic Lymphohistiocytosis. Mayo Clinic Proceedings, 89, 484-492. [Google Scholar] [CrossRef] [PubMed]
[18] Wang, J., Wang, Y., Wu, L., et al. (2016) PEG-Aspargase and DEP Regimen Combination Therapy for Refractory Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocyto-sis. Journal of Hematology & Oncology, 9, Article No. 84. [Google Scholar] [CrossRef] [PubMed]
[19] 程雪, 徐之良. 儿童EB病毒相关性噬血细胞综合征的诊治进展[J]. 医学综述, 2018, 24(18): 3658-3663.
[20] Li, Z., Wang, Y., Wang, J., et al. (2018) Haploidentical Hematopoietic Stem Cell Transplantation for Adult Patients with Ep-stein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis. Leukemia & Lymphoma, 59, 77-84. [Google Scholar] [CrossRef] [PubMed]
[21] Ahn, J.S., Rew, S.Y., Shin, M.G., et al. (2010) Clinical Sig-nificance of Clonality and Epstein-Barr Virus Infection in Adult Patients with Hemophagocytic Lymphohistiocytosis. American Journal of Hematology, 85, 719-722. [Google Scholar] [CrossRef] [PubMed]
[22] Imashuku, S. (2011) Treatment of Epstein-Barr Virus-Related Hemophago-cytic Lymphohistiocytosis (EBV-HLH); Update 2010. Journal of Pediatric Hematology/Oncology, 33, 35-39. [Google Scholar] [CrossRef
[23] 卢象对, 彭建明. EB病毒混合感染的临床分析[J]. 中国实用医药, 2019, 14(15): 69-70.
[24] Johnson, T.S., Terrell, C.E., Millen, S.H., et al. (2014) Etoposide Selectively Ab-lates Activated T Cells to Control the Immunoregulatory Disorder Hemophagocytic Lymphohistiocytosis. The Journal of Immunology, 192, 84-91. [Google Scholar] [CrossRef] [PubMed]
[25] Papageorgiou, S.G., Tsiodras, S., Siakallis, G., et al. (2016) Ep-stein Barr Virus Hemophagocytic Lymphohistiocytosis Related to Rituximab Use and Immunopathogenetic Insights. Pa-thology—Research and Practice, 212, 1194-1198. [Google Scholar] [CrossRef] [PubMed]