血友病B伴腹盆腔假肿瘤一例

张婧; 鞠满凯; 张科民; 陈玲玲; 王玉华; 张磊; 杨仁池; 薛峰

doi:10.12376/j.issn.2097-0501.2022.04.016

血友病B伴腹盆腔假肿瘤一例

doi: 10.12376/j.issn.2097-0501.2022.04.016

1.
中国医学科学院血液病医院 (中国医学科学院血液学研究所) 国家血液系统疾病临床医学研究中心实验血液学国家重点实验室国家血友病病例信息管理中心血栓止血诊疗中心，天津 300020
2.
中国医学科学院血液病医院 (中国医学科学院血液学研究所) 国家血液系统疾病临床医学研究中心实验血液学国家重点实验室国家血友病病例信息管理中心影像科，天津 300020
3.
中国医学科学院血液病医院(中国医学科学院血液学研究所)国家血液系统疾病临床医学研究中心实验血液学国家重点实验室国家血友病病例信息管理中心出凝血实验室，天津 300020

详细信息

通信作者:
薛峰，E-mail: xuefeng@ihcams.ac.cn

中图分类号: R445;R554+.1
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出版历程
- 收稿日期: 2022-08-05
- 录用日期: 2022-08-18
- 网络出版日期: 2022-12-23

A Case of Hemophilia B with Abdominal and Pelvic Pseudotumor

1.
Thrombosis and Hemostasis Center, National Clinical Research Center for Blood Diseases, State Key Laboratory of Experimental Hematology, National Hemophilia Registry Center, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China
2.
Department of Imaging, National Clinical Research Center for Blood Diseases, State Key Laboratory of Experimental Hematology, National Hemophilia Registry Center, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China
3.
Coagulation Laboratory, National Clinical Research Center for Blood Diseases, State Key Laboratory of Experimental Hematology, National Hemophilia Registry Center, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China

More Information

Corresponding author: XUE Feng, E-mail: xuefeng@ihcams.ac.cn

摘要

摘要: 血友病B是一种基因突变导致的凝血因子Ⅸ(FⅨ)缺乏性疾病，主要表现为关节、肌肉及深部组织出血。血友病假肿瘤是反复出血导致液化坏死的软组织形成的肿块，大部分假肿瘤发生在骨骼及肌肉处。本文报道了一例血友病B伴盆腹腔假肿瘤形成的患者，病变位置相对罕见，对患者进行积极有效的止血后，患者血尿症状逐渐好转。该病例提示血友病患者早期及时止血治疗是至关重要的。
- 血友病B /
- 假肿瘤 /
- 影像学表现
Abstract: Hemophilia B is a genetic disorder caused by coagulation factor Ⅸ(FⅨ) deficiency, mainly manifesting as joint, muscle and deep tissue bleeding. Hemophilia pseudotumor is a mass formed by soft tissue liquefaction and necrosis caused by repeated bleeding. Most pseudotumors occur in the bone and muscle. We report a case of hemophilia B with pseudotumor formation in the pelvis and abdomen, where lesion location is relatively rare. After active and effective hemostasis, the patient's hematuria symptom gradually improved. This case suggests that early and timely hemostatic treatment is crucial for patients with hemophilia.
- hemophilia B /
- pseudotumor /
- imaging manifestations
注释:

作者贡献： 张婧总结病例并撰写初稿；鞠满凯收集及分析临床数据；张科民提供影像学资料；陈玲玲收集临床资料数据；王玉华提供检验报告信息；张磊提供临床意见；杨仁池提供临床意见；薛峰初稿审阅及修改，提供临床意见。

利益冲突： 所有作者均声明不存在利益冲突。

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图 1 盆腔MRI结果示假肿瘤压迫膀胱

MRI示膀胱体积小、形态不规整，左侧壁呈鸟嘴状疝入(红色箭头所示)混杂信号的假肿瘤内；T₁WI(A)及T₂WI(B)示疝入膀胱壁明显增厚并与假肿瘤分界不清，腔内可见T₂高信号尿液；膀胱内可见引流管影

Figure 1. Pelvic MRI results showed pseudotumor compression of the bladder

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图 2 腹部增强CT结果

CT可见混杂信号肿物压迫邻近左髂总动脉(红色箭头所示)

Figure 2. Abdominal contrast enhancement CT scan results

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图 3 膀胱镜结果可见3个窦口与膀胱外肿物相通

Figure 3. Cystoscopy results showed that 3 sinus mouths communited with the extravesical nass

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图 4 腹部增强CT示假肿瘤矢状位

矢状位假肿瘤长轴约21.5 cm(红色箭头所示)

Figure 4. Abdominal contrast enhancement CT scan showed pseudotumor in sagittal view

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图 5 腹部增强CT示假肿瘤冠状位

冠状位假肿瘤长轴约20.9 cm(红色箭头所示)

Figure 5. Abdominal contrast enhancement CT scan showed pseudotumor in coronal view

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