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成人免疫性血小板减少性紫癜合并肝血管瘤1例报告并文献复习OA北大核心CSTPCD

Adult immune thrombocytopenia combined with hepatic hemangioma:a case report and literature review

中文摘要英文摘要

背景与目的:成人免疫性血小板减少性紫癜(ITP)合并肝血管瘤临床罕见,容易误诊误治.笔者报告的1例成人ITP合并肝血管瘤患者的诊治经过,以期为临床提供参考和借鉴. 方法:回顾性分析桂林医学院第二附属医院收治的1例成人ITP合并肝血管瘤患者的临床资料,并结合文献总结该病的临床诊治特点. 结果:患者为46岁女性,因"全身皮肤黏膜出血1个月余,血小板减少1d"入住血液内科.血细胞分析血小板计数4.0×109/L,凝血功能正常,骨髓穿刺活检提示巨核细胞相对增多,血小板少见,高度疑似ITP.经激素等治疗后皮肤黏膜出血好转,血小板升高至59 × 109/L.上腹部增强CT提示右肝巨大血管瘤,转至肝胆胰外科行肝血管瘤切除,术后术区渗血明显,阴道流血,血小板逐步降低至3.0×109/L,右侧胸腔大量积液,但凝血功能正常.经止血、输血、调节免疫、抗感染、胸腔穿刺引流处理后逐步恢复.术后第13天患者血小板升高至220 × 109/L.随访32个月,患者皮肤黏膜不再出血,血小板计数维持在正常范围. 结论:成人ITP,需警惕合并肝血管瘤,处理血管瘤可使患者获得良好的远期效果,但需做好围术期管理以使患者顺利恢复.

Background and Aims:Adult immune thrombocytopenia(ITP)combined with hepatic hemangioma is clinically rare and prone to misdiagnosis and mistreatment.Here,the authors report the diagnosis and treatment process of a case of adult ITP combined with hepatic hemangioma,aiming to provide reference and insights for clinical practice. Methods:A retrospective analysis of the clinical data of an adult patient with ITP and concomitant hepatic hemangioma admitted to the Second Affiliated Hospital of Guilin Medical College was conducted.The clinical features and treatment characteristics of the disease were summarized in conjunction with relevant literature. Results:The patient,a 46-year-old female,was admitted to the Department of Hematology due to generalized skin and mucosal bleeding for over a month,and thrombocytopenia for 1 d.Blood cell analysis revealed a platelet count of 4.0x109/L,normal coagulation function,and bone marrow aspiration biopsy suggested a relative increase in megakaryocytes with rare platelets.The diagnosis was highly suspicious of ITP.After treatment with steroids and other measures,the skin and mucosal bleeding improved,and the platelet count increased to 59×109/L.Abdominal enhanced CT indicated a massive hemangioma on the right liver,and then,the patient was transferred to the Department of Hepatobiliary and Pancreatic Surgery for resection of the liver hemangioma.After operation,there was significant bleeding in the surgical area,vaginal bleeding,and a gradual decrease in platelets to 3.0×109/L.The right pleural cavity accumulated a large amount of fluid,but coagulation function remained normal.After interventions including hemostasis,transfusion,immune modulation,anti-infection measures,and thoracic puncture drainage,the patient gradually recovered.On the 13th d after operation,the platelet count increased to 220×109/L.Follow-up for 32 months revealed no recurrence of skin and mucosal bleeding,with the platelet count maintained within the normal range. Conclusion:In adult ITP,vigilance is necessary for the presence of concomitant hepatic hemangioma.Treatment of the hemangioma can achieve favorable long-term outcomes,but meticulous perioperative management is essential for the uneventful recovery of patients.

吴嘉兴;邓维沛;赵东康;蒋建晖;张俊艳;姚红兵

桂林医学院第二附属医院肝胆胰外科,广西桂林 541199桂林医学院第二附属医院血液内科,广西桂林 541199

临床医学

肝肿瘤血管瘤紫癜,血小板减少性Kasabach-Merritt综合征肝切除术

Liver NeoplasmsHemangiomaPurpura,ThrombocytopenicKasabach-Merritt SyndromeHepatectomy

《中国普通外科杂志》 2024 (001)

44-51 / 8

广西壮族自治区卫生健康委员会自筹经费科研课题基金资助项目(Z20190035);广西壮族自治区医疗卫生重点培育学科建设项目基金资助项目(桂卫科教发[2021]8号).

10.7659/j.issn.1005-6947.2024.01.006

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