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
作者:
通讯作者:
作者单位:

1.桂林医学院第二附属医院 肝胆胰外科,广西 桂林 541199;2.桂林医学院第二附属医院 血液内科,广西 桂林 541199

作者简介:

吴嘉兴,桂林医学院第二附属医院副主任医师,主要从事肝脏肿瘤方面的研究。

基金项目:

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)。


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

1.Department of Hepatobiliary and Pancreatic Surgery, the Second Affilicated Hospital of Guilin Medical University, Guilin, Guangxi 541199, China;2.Department of Hematology, the Second Affilicated Hospital of Guilin Medical University, Guilin, Guangxi 541199, China

Fund Project:

  • 摘要
  • |
  • 图/表
  • |
  • 访问统计
  • |
  • 参考文献
  • |
  • 相似文献
  • |
  • 引证文献
  • |
  • 资源附件
  • |
  • 音频文件
  • |
  • 视频文件
    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1 d”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。

    Abstract:

    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 4.0×109/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.

    表 1 患者术后第5天胸腔积液常规及生化结果Table 1 Routine and biochemical results of pleural fluid on postoperative day 5 of the patient
    表 2 患者血小板及凝血功能变化Table 2 Changes in platelet and coagulation function of the patient
    图1 骨髓活检病理 A:骨髓增生活跃,粒系占65%、红系占19%,粒∶红=3.42∶1,瑞氏染色(×1 000);B:粒系增生活跃,各阶段细胞比值及形态大致正常,红系增生活跃,以中、晚幼红细胞增生为主,形态未见明显异常,成熟红细胞大致正常,铁染色(×400);C:淋巴细胞、浆细胞大致正常(阅全片见巨核细胞84个,可见多分叶核巨核细胞,免疫组化染色未见微巨核细胞,血小板少见),瑞氏染色(×1 000);D:内铁(-),外铁(±),铁染色(×400)Fig.1 Bone marrow biopsy A: Active bone marrow proliferation with the granulocytic series accounting for 65%, the erythroid series for 19%, and a granulocyte-to-erythroid ratio of 3.42∶1, Wrights staining(×1 000); B: Active proliferation of granulocytic series with normal cell ratios and morphology in various stages, and active proliferation of erythroid series, mainly with an increase in intermediate and late-stage erythroblasts, with no apparent morphological abnormalities, and generally normal mature red blood cells, Iron staining (×400); C: Lymphocytes and plasma cells generally normal (the entire slide examination observed 84 megakaryocytes that included the polylobulated megakaryocytes, and no micromegakaryocytes and rare platelets were observed by immunohistochemical staining), Wrights staining(×1 000); D: Iron staining (×400) showing (-) for internal iron and (±) for extracellular iron
    图2 上腹部增强CT示肝Ⅵ、Ⅶ段见12.0 cm×9.8 cm×8.0 cm肿瘤(箭头示肿瘤) A:平扫期肿瘤呈类圆形,边界清晰,密度均匀,中心处为缺血坏死灶;B:动脉期肿瘤边缘近环状强化,密度与主动脉接近;C:静脉期肿瘤内造影剂向心性扩展Fig.2 Enhanced CT of the upper abdomen showing a 12.0 cm × 9.8 cm × 8.0 cm tumor in segments Ⅵ and Ⅶ of the liver (indicated by the arrow) A: A nearly circular tumor with clear borders, uniform density, and a central area of ischemic necrosis in the non-contrast phase; B: A nearly annular enhancement of the tumor at the periphery, with density similar to the adjacent main artery in the arterial phase; C: Centripetal expansion of the contrast agent within the tumor in the venous phase
    图3 肝组织切除术后大体标本(箭头示肝血管瘤) A:标本外面观;B:标本剖面观Fig.3 Gross specimen after hepatectomy (hemangioma indicated by the arrow) A: External view of the specimen; B: Cross-sectional view of the specimen
    图4 术后病理示肿瘤由大小不一扩张、薄壁的血管腔构成,内衬单层扁平上皮,细胞无异性和核分裂,肿瘤性血管腔由包含小血管的纤维间隔分开(HE×200)Fig.4 Postoperative pathological image showing the tumor composed of variably sized, dilated, thin-walled vascular lumina lined by a single layer of flat epithelium, with no atypia or nuclear division, and the neoplastic vascular lumens separated by fibrous septa containing small blood vessels (HE×200)
    参考文献
    相似文献
    引证文献
引用本文

吴嘉兴,邓维沛,赵东康,蒋建晖,张俊艳,姚红兵.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[J].中国普通外科杂志,2024,33(1):44-51.
DOI:10.7659/j. issn.1005-6947.2024.01.006

复制
分享
文章指标
  • 点击次数:
  • 下载次数:
历史
  • 收稿日期:2023-02-27
  • 最后修改日期:2023-06-24
  • 录用日期:
  • 在线发布日期: 2024-02-05