中国继续医学教育
中國繼續醫學教育
중국계속의학교육
China Continuing Medical Education
2015年
25期
143-144
,共2页
鼻神经胶质细胞瘤%血友病 A
鼻神經膠質細胞瘤%血友病 A
비신경효질세포류%혈우병 A
Nasal glioma cells%Hemophilia A
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 F Ⅷ/F Ⅸ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。
目的：通過分析1例鼻神經膠質細胞瘤閤併血友病 A 患者的臨床資料併複習相關文獻，提高對該病的認識。方法結閤1例鼻神經膠質細胞瘤閤併血友病 A 患者行鼻腔腫物切除術的臨床資料及文獻報道，對本病的髮病機製、臨床錶現、診斷和治療進行討論。結果鼻神經膠質細胞瘤是罕見的先天性神經組織的“良性腫瘤”，多繫嬰幼兒，往往齣生後不久就被髮現，主要治療方法是手術切除。血友病是一種遺傳性齣血性疾病，是由于 F Ⅷ/F Ⅸ基因突變所引起的 X-連鎖隱性遺傳性疾病，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），患兒絕大多數為男童。本例為一3歲8月男性鼻腔腫物患者，自幼即髮現其右側鼻腔有腫物，腫物隨年齡增長而增大。術前 APTT 延長，進一步檢查髮現血漿凝血因子Ⅷ活性下降併被診斷為血友病A。術後輸註人凝血因子Ⅷ以改善凝血功能，術腔恢複好，無活動性滲齣血。病理結果示鼻神經膠質細胞瘤。結論鼻神經膠質細胞瘤閤併血友病 A 臨床罕見，術前明確診斷及術後及時補充所缺乏的因子，是十分關鍵的環節。
목적：통과분석1례비신경효질세포류합병혈우병 A 환자적림상자료병복습상관문헌，제고대해병적인식。방법결합1례비신경효질세포류합병혈우병 A 환자행비강종물절제술적림상자료급문헌보도，대본병적발병궤제、림상표현、진단화치료진행토론。결과비신경효질세포류시한견적선천성신경조직적“량성종류”，다계영유인，왕왕출생후불구취피발현，주요치료방법시수술절제。혈우병시일충유전성출혈성질병，시유우 F Ⅷ/F Ⅸ기인돌변소인기적 X-련쇄은성유전성질병，포괄혈우병 A（유우응혈인자Ⅷ결핍）화혈우병 B（유우응혈인자Ⅸ결핍），환인절대다수위남동。본례위일3세8월남성비강종물환자，자유즉발현기우측비강유종물，종물수년령증장이증대。술전 APTT 연장，진일보검사발현혈장응혈인자Ⅷ활성하강병피진단위혈우병A。술후수주인응혈인자Ⅷ이개선응혈공능，술강회복호，무활동성삼출혈。병리결과시비신경효질세포류。결론비신경효질세포류합병혈우병 A 림상한견，술전명학진단급술후급시보충소결핍적인자，시십분관건적배절。
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.
