病例报告：一例伴有自发性巨大腹膜后血肿的AL淀粉样变性病例。
Case report: a case of AL amyloidosis with spontaneous giant retroperitoneal hematoma.
作者信息
Sheng Yaqi, Zhu Huadong
机构信息
Emergency Department, The State Key Laboratory for Complex, Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
出版信息
Int J Emerg Med. 2024 Dec 27;17(1):200. doi: 10.1186/s12245-024-00801-3.
BACKGROUND
Systemic amyloidosis is a kind of clinical syndrome in which amyloid is deposited between the cells of various organs in the body, resulting in gradual failure of the function of the affected organs. Depending on the site of amyloid deposition, it may show various clinical symptoms of multiple system involvement.
PATIENT CONCERNS
A 44-years-old female with spontaneous giant retroperitoneal hematoma was admitted to the emergency department of Peking Union Medical College Hospital in Mar 2023.
DIAGNOSES
She was found with a extremely X-factor deficiency and diagnosed with AL amyloidosis according to pathological findings finally.
INTERVENTIONS AND OUTCOMES
She received a variety of treatments to improve her coagulation function and underwent chemotherapy for AL in the hematology department which improved her coagulation function and was discharged to her local hospital for follow-up treatment.
CONCLUSION
This case provides a new reference for emergency doctors in the diagnosis and treatment of acute severe hemorrhagic diseases.
背景
系统性淀粉样变性是一种临床综合征，其中淀粉样蛋白沉积于身体各器官的细胞之间，导致受影响器官的功能逐渐衰竭。根据淀粉样蛋白沉积的部位，它可能表现出多系统受累的各种临床症状。
患者情况
一名44岁女性因自发性巨大腹膜后血肿于2023年3月入住北京协和医院急诊科。
诊断
发现她存在极罕见的X因子缺乏，并最终根据病理结果诊断为AL型淀粉样变性。
干预措施及结果
她接受了多种改善凝血功能的治疗，并在血液科接受了AL型化疗，凝血功能得到改善，随后出院至当地医院进行后续治疗。
结论
该病例为急诊医生诊断和治疗急性重症出血性疾病提供了新的参考。
Zotero 插件