鼻神经胶质细胞瘤合并血友病A1例并文献复习  

1 Case Report of Nasal Glioma Cells Merge With Hemophilia A and Literature Review

作　　者：王燕楠[1] 王素芳[1] 韩富根[1] 

机构地区：[1]河南省郑州市儿童医院耳鼻喉科,450000

出　　处：《中国继续医学教育》2015年第25期143-144,共2页China Continuing Medical Education

摘　　要：目的通过分析1例鼻神经胶质细胞瘤合并血友病A患者的临床资料并复习相关文献,提高对该病的认识。方法结合1例鼻神经胶质细胞瘤合并血友病A患者行鼻腔肿物切除术的临床资料及文献报道,对本病的发病机制、临床表现、诊断和治疗进行讨论。结果鼻神经胶质细胞瘤是罕见的先天性神经组织的"良性肿瘤",多系婴幼儿,往往出生后不久就被发现,主要治疗方法是手术切除。血友病是一种遗传性出血性疾病,是由于FⅧ/FⅨ基因突变所引起的X-连锁隐性遗传性疾病,包括血友病A(由于凝血因子Ⅷ缺乏)和血友病B(由于凝血因子Ⅸ缺乏),患儿绝大多数为男童。本例为一3岁8月男性鼻腔肿物患者,自幼即发现其右侧鼻腔有肿物,肿物随年龄增长而增大。术前APTT延长,进一步检查发现血浆凝血因子Ⅷ活性下降并被诊断为血友病A。术后输注人凝血因子Ⅷ以改善凝血功能,术腔恢复好,无活动性渗出血。病理结果示鼻神经胶质细胞瘤。结论鼻神经胶质细胞瘤合并血友病A临床罕见,术前明确诊断及术后及时补充所缺乏的因子,是十分关键的环节。Objective By analyzing the clinical data of one case of nasal glioma cells in patients with hemophilia A and review relevant literature, raise awareness of the disease. Methods Combined with one case of nasal glioma cells merge with hemophilia A patients with nasal tumor resection and clinical data reported in the literature on the pathogenesis of the disease, clinical manifestations, diagnosis and treatment will be discussed. Results Nasal glial cell tumors are rare congenital neural tissue, “benign”, mostly the infants, often shortly after birth was found that the main treatment is surgical excision.Hemophilia is a hereditary bleeding disorder, is due F Ⅷ/ F Ⅸ mutations caused by X- linked recessive genetic disorders, including hemophilia A （due to a lack of coagulation factor Ⅷ） and hemophilia B （due to lack of clotting factor Ⅸ ）, the vast majority of children with boys.In this case, a 3 years and 8 months nasal tumor patient, a child that is found to the right nasal tumor, tumor increases with age.Preoperative APTT prolonged, further examination revealed decreased activity of the plasma clotting factorⅧ was diagnosed with hemophilia A.After infusion of human coagulation factor Ⅷ to improve coagulation, surgical cavity recovery, and no active oozing blood.Pathology results are shown nasal glioma cells. Conclusion Nasal glioma cells merge hemophilia A rare clinical preoperative and postoperative diagnosis replenish lacking factor is crucial link.

关 键 词：鼻神经胶质细胞瘤 血友病A 

分 类 号：R554[医药卫生—血液循环系统疾病]