hypersplenism

脾功能亢进
  • 文章类型: Case Reports
    脾动脉栓塞术在治疗各种病因非创伤性的内科和外科疾病中起着重要作用。除了其在治疗脾创伤中的公认和广泛讨论的作用。在源自脾或脾动脉的灾难性出血的非创伤性紧急情况中,脾动脉栓塞术作为一种明确的治疗方法可以有效地实现止血,临时稳定措施,或术前优化技术。除了紧急的临床条件,脾动脉栓塞术可作为脾切除术的替代治疗脾功能亢进患者的选择。在这里,我们报告了在我们中心进行的6例脾动脉栓塞术,以强调其各种适应症。本文旨在证明脾动脉栓塞在不同临床情况下的作用以及通过说明性病例采用的技术背后的注意事项。
    Splenic artery embolization plays an important role in the management of various medical and surgical conditions that are non-traumatic in etiology, in addition to its well-established and widely discussed role in managing splenic trauma. In nontraumatic emergencies of catastrophic bleeding originating from the spleen or splenic artery, splenic artery embolization can be effective in achieving hemostasis as a definitive management, temporary stabilizing measure, or preoperative optimization technique. In addition to emergency clinical conditions, splenic artery embolization can be performed electively as an alternative to splenectomy for managing patients with hypersplenism. Herein, we report 6 cases of splenic artery embolization performed at our center to highlight its various indications. This article aims to demonstrate the role of splenic artery embolization in different clinical scenarios and the considerations behind the techniques employed through illustrative cases.
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  • 文章类型: Case Reports
    部分脾动脉栓塞术(PSAE)是治疗门静脉高压继发脾功能亢进的一种替代治疗方式。我们正在介绍一系列因症状性脾功能亢进而接受PSAE治疗的门静脉高压症患者。
    我们纳入了2022年1月至2022年12月接受PSAE的门脉高压患者。记录患者特征和手术相关并发症。分析数据以改善血液学参数。
    总共11名(7名女性,中位年龄34[18-56]岁)的患者被包括在内。3例患者为肝硬化(乙型肝炎-2,代谢功能障碍相关的脂肪变性肝病-1),8例为非肝硬化(肝外门静脉阻塞-5,非肝硬化门静脉纤维化-3)。5例同时存在脾动脉瘤。在所有情况下都取得了技术成功。栓塞后,血红蛋白,白细胞和血小板计数在4周改善,12周和24周伴随症状改善。所有患者均有栓塞后综合征。一名患者出现了暂时性腹水和继发性细菌性腹膜炎,并进行了保守治疗。一名患者因脾脓肿和败血症死亡。
    虽然,血液学参数和症状改善术后,PSAE与主要并发症相关,应仅在选定病例中审慎进行。图形抽象如图1所示。
    UNASSIGNED: Partial splenic artery embolization (PSAE) is an alternative treatment modality for managing hypersplenism secondary to portal hypertension. We are presenting a case series of patients with portal hypertension who underwent PSAE for symptomatic hypersplenism.
    UNASSIGNED: We included patients with portal hypertension who underwent PSAE from January 2022 to December 2022. Patients\' characteristics and procedure related complications were noted. Data were analyzed for improvement in the hematological parameters.
    UNASSIGNED: A total of 11 (7 women, median age 34 [18-56] years) patients were included. Three patients were cirrhotic (hepatitis B-2, metabolic dysfunction-associated steatotic liver disease -1) and 8 were non-cirrhotic (extra-hepatic portal vein obstruction-5, Non cirrhotic portal fibrosis-3). Splenic artery aneurysm was concomitantly present in 5 cases. Technical success was achieved in all cases. Post embolization, hemoglobin, white blood cells and platelet counts improved at 4 weeks, 12 weeks and 24 weeks along with symptomatic improvement. All patients had post-embolization syndrome. One patient developed transient ascites and secondary bacterial peritonitis which was managed conservatively. One patient died due to splenic abscess and septicemia.
    UNASSIGNED: Although, hematological parameters and symptoms improve post procedure, PSAE is associated with major complications and should be performed judiciously in selected cases only. Graphical abstract is presented in Figure 1.
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  • 文章类型: Case Reports
    妊娠合并巨大脾肿大是一种罕见的临床实体,会带来特殊的困难,比如贫血,血小板减少症,腹水,和黄疸。1例妊娠合并大脾肿大、全血细胞减少的病例报告强调了及时诊断和有效治疗的价值。脾肿大有很多原因,包括病毒感染,血液学问题,门静脉高压症,和代谢异常。一名妊娠37周时有大量脾肿大的29岁孕妇入院,并接受了剖宫产术,以避免脾肿大的并发症。该病例报告讨论了妊娠期间巨大脾肿大引起的产科管理困难,包括交货方式的选择。严重的并发症包括脾破裂和出血,特别是当存在全血细胞减少时。需要多次输血,输血治疗的潜在副作用,本病例报告讨论了评估母胎结局时与脾肿大起源相关的因素。该研究得出的结论是,在怀孕期间出现全血细胞减少脾肿大的病例中,警惕监测,及时干预,多学科方法对于母亲和胎儿取得积极成果至关重要。
    Massive splenomegaly complicating pregnancy is a rare clinical entity that poses special difficulties, such as anemia, thrombocytopenia, ascites, and jaundice. This case report of a pregnant woman with large splenomegaly and pancytopenia highlights the value of prompt diagnosis and effective treatment. Splenomegaly can have a number of causes, including viral infections, hematological problems, portal hypertension, and metabolic abnormalities. A 29-year-old gravida 3 woman at 37 weeks of gestation who had massive splenomegaly was admitted and underwent a cesarean section to avoid complications of splenomegaly. The case report discusses the difficulties in obstetric management caused by enormous splenomegaly during pregnancy, including the choice of delivery method. Significant complications include splenic rupture and bleeding, particularly when pancytopenia is present. The need for several transfusions, the potential side effects of transfusion therapy, and factors related to the origin of splenomegaly when assessing maternal-fetal outcomes are discussed in this case report. The study concludes that in cases with pancytopenia splenomegaly during pregnancy, vigilant monitoring, prompt intervention, and a multidisciplinary approach are crucial to achieve positive outcomes for both the mother and the fetus.
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  • 文章类型: Case Reports
    背景:非肝硬化门静脉高压症(NCPH)的特征是肝脏没有肝硬化修饰,门静脉和肝静脉通畅。与普通人群相比,NCPH与怀孕期间孕产妇和围产期发病率和死亡率的风险增加有关。NCPH存在于大多数(74.1%)患有门静脉高压症的孕妇中。每4例妊娠中就有1例(25%)在怀孕期间并发静脉曲张出血。到目前为止,对于这种罕见疾病的治疗,世界上仍然没有共识。
    方法:我们具体说明了一个罕见的例子,患者在8岁时被诊断为NCPH和脾功能亢进,并且在引产期间由于高血压导致的首次妊娠流产而经历了3L大出血。
    方法:诊断先兆早产伴宫颈扩张,妊娠期糖尿病,脾肿大伴脾功能亢进,门静脉高压症,肾功能受损的肾脏实质损害导致妊娠29+3周时无脾切除的剖宫产分娩。
    结果:她和她的孩子在手术后3个月都处于良好状态。
    结论:孕前咨询,正在进行的后续行动,监测对NCPH孕妇至关重要。多学科团队方法,通过及时干预和深入监测,可以帮助在合并门静脉高压的妊娠中获得最佳的孕产妇-围产期结局。我们的病例提供了成功的治疗,但需要更多的NCPH管理指南。
    BACKGROUND: Non-cirrhotic portal hypertension (NCPH) is characterized by the absence of cirrhotic modification of the liver and the patency of the portal and hepatic veins. When compared to the general population, NCPH is associated with an increased risk of maternal and perinatal morbidity and mortality during pregnancy. NCPH was present in the majority (74.1%) of pregnant women with portal hypertension. One (25%) out of every 4 pregnancies was complicated by variceal hemorrhage while pregnant. So far, there is still no consensus in the world about the treatment of this rare condition.
    METHODS: We have specifically illustrated a rare instance where the patient was diagnosed with NCPH and hypersplenism at the age of 8 and experienced a 3 L massive hemorrhage during labor induction as a result of her first pregnancy loss due to hypertension.
    METHODS: The diagnosis of threatened preterm labor with cervical dilatation, gestational diabetes mellitus, massive splenomegaly with hypersplenism, portal vein hypertension, and parenchymal damage of kidney with impaired renal function led to the cesarean delivery of the second pregnancy at 29+3 weeks gestation without splenectomy after been evaluated by multispecialty team.
    RESULTS: She and her child were both in generally good condition 3 months after the operation.
    CONCLUSIONS: Preconception counseling, ongoing follow-up, and monitoring are crucial in pregnant women with NCPH. A multidisciplinary team approach, with timely intervention and intensive monitoring, can help achieve optimal maternal-perinatal outcomes in pregnancies complicated with portal hypertension. Our case provided a successful treatment, but more guidelines for the management of NCPH are needed.
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  • 文章类型: Case Reports
    UNASSIGNED: Splenic artery aneurysm is one of the most common visceral aneurysms. Patients are usually asymptomatic. Splenic artery aneurysm if untreated has the potential for rupture and is therefore life-threatening. Its association with extrahepatic portal vein obstruction is rare.
    METHODS: A 25-year female was incidentally diagnosed with a splenic artery aneurysm with extrahepatic portal vein obstruction with splenomegaly 8 years back during the 5th month of her second pregnancy. No intervention was done back then. Recently, she presented to the surgical gastroenterology outpatient department with an increasing abdominal mass. On examination, the patient was pale and splenomegaly was present. Hematological reports were suggestive of hypersplenism. The patient underwent splenectomy and aneurysmal resection with a proximal splenorenal shunt as the best course of treatment.
    CONCLUSIONS: Due to the rarity of the disease, the management is still challenging and needs further study. Diagnosis can be made clinically with support from imaging modalities. Surgical treatment has a good outcome in such patients. Even with the availability of less invasive procedures such as endovascular treatment, open surgery is preferred.
    CONCLUSIONS: Proximal splenorenal shunt is a well-accepted surgical procedure for extrahepatic portal vein obstruction. Splenectomy and aneurysmal resection can relieve hypersplenism and treat splenic artery aneurysm in patients with isolated splenic artery aneurysm at the splenic hilum.
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  • 文章类型: Journal Article
    UNASSIGNED:目的比较开放射频消融术联合脾切除和心包断流术与肝移植治疗肝癌合并门静脉高压和脾功能亢进患者的短期和长期治疗效果。
    未经评估:在研究期间,连续的肝癌门脉高压和脾功能亢进患者的治疗结果,将脾切除术和心包血管离断术(研究组)与接受肝移植的HCC患者的病例匹配对照组的治疗结果进行比较.
    未经评估:研究组由32名患者组成,对照组包括从155例肿瘤大小匹配的患者中选择的32例患者,年龄,性别,MELD疼痛,肿瘤位置,TNM分类,脾肿大程度和Child-Pugh分期。两组的术前实验室检查和肿瘤特征的基线数据具有可比性。研究组和对照组的平均随访时间分别为43.2±5.3个月和44.9±5.8个月,分别。尽管对照组的无病生存率优于研究组(P<0.001)。两组患者累积总生存时间和门静脉血栓形成发生率差异无统计学意义(P=0.670,0.083).与对照组相比,研究组术中出血量明显减少,术后胸腔积液和肺炎的发生率较低(均P<0.05)。
    未经评估:开放式射频消融,对于肝供体短缺的部分精心挑选的患者,脾切除术和心包血管离断术治疗小肝癌合并门脉高压和脾功能亢进可作为替代疗法.
    UNASSIGNED: To compare the short- and long-term treatment outcomes of open radiofrequency ablation combined with splenectomy and pericardial devascularization versus liver transplantation for hepatocellular carcinoma patients with portal hypertension and hypersplenism.
    UNASSIGNED: During the study period, the treatment outcomes of consecutive HCC patients with portal hypertension and hypersplenism who underwent open radiofrequency ablation, splenectomy and pericardial devascularization (the study group) were compared with the treatment outcomes of a case-matched control group of HCC patients who underwent liver transplantation.
    UNASSIGNED: The study group consisted of 32 patients, and the control group comprised 32 patients selected from 155 patients who were case-matched by tumor size, age, gender, MELD sore, tumor location, TNM classification, degree of splenomegaly and Child-Pugh staging. Baseline data on preoperative laboratory tests and tumor characteristics were comparable between the two groups. The mean follow-up was 43.2 ± 5.3 months and 44.9 ± 5.8 months for the study and control groups, respectively. Although the disease-free survival rates of the control group were better than those of the study group (P < 0.001), there was no significant difference in the cumulative overall survival time or the incidence of portal vein thrombosis between the two groups (P = 0.670, 0.083). Compared with the control group, the study group had significantly less intraoperative blood loss, and lower incidences of postoperative pleural effusion and pneumonia (all P < 0.05).
    UNASSIGNED: Open radiofrequency ablation, splenectomy and pericardial devascularization for small HCCs with portal hypertension and hypersplenism can be an alternative therapy for a subset of carefully selected patients under the shortage of liver donors.
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  • 文章类型: Journal Article
    脾功能亢进是儿童肝硬化或门静脉海绵体瘤继发的门静脉高压和脾肿大的结果。为了避免肝移植(LT)或静脉分流(VS)后持续的脾功能亢进和脾肿大,部分脾切除术(PS)可能是相关的治疗选择。这项回顾性研究的目的是评估出现脾功能亢进的儿童的PS结果。
    评估了以下终点:(1)脾功能亢进的逆转及其随时间的持久性,(2)术后转归,(3)脾脏大小和体积的疗程和(4)与未进行PS的对照组的比较。
    在1996年至2020年之间,有16名儿童接受了肝硬化LT(8例)或门脉海绵体瘤VS(8例)相关的PS。从第0天到第1个月,平均血小板和白细胞计数(WBC)分别从第0天的48±19天到176±70×109/L(P<0.0001)和从2469±853到7198±3982/L(P=0.001)。PS使脾脏长度和体积分别从176±33到112±24cm(P<0.0001)和从1228±464到450±297cm3(P=0.0003)显着减少。平均随访92.6±84.7个月(范围:4.1-210.7),14例患者存活,血小板和白细胞计数正常,脾脏大小持续减小。与对照组相比,PS与从基线到一年的显著血小板计数升高相关。
    PS与LT或VS联合治疗脾功能亢进和脾肿大似乎是有效的,而不影响预后。
    Hypersplenism is a consequence of portal hypertension and splenomegaly secondary to cirrhosis or portal cavernoma in children. In order to avoid persistent hypersplenism and splenomegaly after liver transplantation (LT) or venous shunt (VS), partial splenectomy (PS) may represent a relevant therapeutic option. The aim of this retrospective study was to evaluate the results of PS performed in children presenting hypersplenism.
    The following end-points were evaluated: (1) reversion of hypersplenism and its durability over time, (2) postoperative outcome, (3) courses of spleen size and volume and (4) comparison to a control group in which PS was not performed.
    Between 1996 and 2020, 16 children underwent PS associated with LT (8 cases) for cirrhosis or VS (8 cases) for portal cavernoma. From Day 0 to 1 month, mean platelet and white blood cell counts (WBC) dramatically improved from 48 ± 19 at day 0 to 176 ± 70 × 109/L (P < 0.0001) and from 2469 ± 853 to 7198 ± 3982/L (P = 0.001) respectively. PS allowed significant reduction of splenic length and volume from 176 ± 33 to 112 ± 24 cm (P < 0.0001) and from 1228 ± 464 to 450 ± 297 cm3 (P = 0.0003) respectively. After a mean follow-up of 92.6 ± 84.7 months (range: 4.1-210.7), 14 patients are alive with normal platelet and WBC counts and persistent spleen size reduction. Compared to control group, PS was associated with a significant platelet count rise from baseline to one year.
    PS appears to be effective for treatment of hypersplenism and splenomegaly in combination with LT or VS without compromising outcome.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    具有小部分肝移植的成人对成人活体供体肝移植通常需要术中门静脉流入调节,以防止门静脉过度灌注和随后的小体积综合征(SFSS)。然而,人们担心这些调制技术的具体发病率。本研究旨在通过辅助部分原位肝移植(APOLT)同时进行次全脾切除术,降低终末期肝硬化患者的灌注后门静脉压力并纠正严重的脾功能亢进。一名29岁的男子被诊断出患有隐源性肝硬化和严重的门脉高压,复发性急性静脉曲张破裂出血,严重的血小板减少症,入院前还有大量腹水.受体的左肝切除后,我们使用他51岁父亲的左叶移植物进行APOLT,移植物与受体的重量比为0.55%。术中,同时进行脾次全切除术,以降低移植物灌注后门静脉压力低于15mmHg,并纠正重度脾功能亢进相关的全血细胞减少.受者术后住院过程顺利,未发生SFSS和手术相关并发症。术后血小板和白细胞计数保持在正常范围内。活体供者术后6天出院,恢复良好,无并发症。经过35.3个月的随访,受者和供者均生活在良好的肝功能和整体状况下。这是APOLT期间使用小型活体捐赠的左肝叶同时进行次全脾切除术的首例报告。在某些终末期肝硬化成年患者中,这被证明是调节门静脉流入和纠正严重脾功能亢进的可行方法。对于选择的终末期肝硬化成年患者,使用小型肝移植的APOLT可能是安全可行的治疗选择。
    Adult-to-adult living donor liver transplantation with small partial liver grafts often requires intraoperative portal inflow modulation to prevent portal hyperperfusion and subsequent small-for-size syndrome (SFSS). However, there are concerns about the specific morbidity of these modulation techniques. This study aims to lower post-perfusion portal venous pressure and correct severe hypersplenism in a patient with end-stage liver cirrhosis by simultaneous subtotal splenectomy during auxiliary partial orthotopic liver transplantation (APOLT). A 29-year-old man was diagnosed with cryptogenic cirrhosis and severe portal hypertension suffered recurrent acute variceal bleeding, severe thrombocytopenia, and massive ascites before admission to our hospital. After the recipient\'s left liver was resected, we performed APOLT using his 51-year-old father\'s left lobe graft with a graft-to-recipient weight ratio of 0.55%. Intraoperatively, simultaneous subtotal splenectomy was performed to lower graft post-perfusion portal vein pressure below 15 mmHg and correct severe hypersplenism-related pancytopenia. The recipient\'s postoperative hospital course was uneventful with no occurrence of SFSS and procedure-related complications. Platelet and leukocyte counts remained in the normal ranges postoperatively. The living donor was discharged 6 days after the operation and recovered well-with no complications. After a follow-up period of 35.3 months, both the recipient and donor live with good liver function and overall condition. This is the first case report of simultaneous subtotal splenectomy during APOLT using small-for-size living-donated left liver lobes, which is demonstrated to be a viable procedure for modulating portal inflow and correcting severe hypersplenism in selected adult patients with end-stage liver cirrhosis. APOLT using a small-for-size liver graft may be a safe and feasible treatment option for selected adult patients with end-stage liver cirrhosis.
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  • 文章类型: Case Reports
    背景:脾功能亢进导致血小板减少症,这可能导致化疗的减少或停止。部分脾栓塞术(PSE)是治疗血小板减少合并脾功能亢进的有效方法。然而,目前还没有关于胃癌患者在PSE治疗与肿瘤浸润相关的脾脾功能亢进后恢复并继续化疗的报道.这里,我们报告了2例,其中我们对侵犯脾静脉的胃癌相关脾功能亢进进行了PSE治疗.两种情况下都继续化疗。
    方法:两名患者均因晚期胃癌导致血小板减少伴脾肿大,需要停止化疗。
    方法:上消化道内镜和计算机断层扫描显示晚期胃癌侵犯脾静脉和脾肿大。两名患者均出现血小板减少症。
    方法:患者接受PSE治疗。
    结果:PSE导致血小板计数增加,化疗可以恢复。
    结论:PSE似乎是治疗晚期胃癌合并脾肿大的血小板减少症的有效方法,并且可以继续化疗。
    BACKGROUND: Hypersplenism causes thrombocytopenia, which may lead to the reduction or discontinuation of chemotherapy. Partial splenic embolization (PSE) is an effective treatment for thrombocytopenia associated with hypersplenism. However, there have been no reports of patients with gastric cancer who have resumed and continued chemotherapy after PSE for splenic hypersplenism associated with tumor infiltration.Here, we report two cases in which we performed PSE for hypersplenism associated with gastric cancer that had invaded the splenic vein. Chemotherapy was continued in both cases.
    METHODS: Both patients developed thrombocytopenia with splenomegaly due to advanced gastric cancer that required discontinuation of chemotherapy.
    METHODS: Upper gastrointestinal endoscopy and computed tomography showed advanced gastric cancer with invasion of the splenic vein and splenomegaly. Both patients developed thrombocytopenia.
    METHODS: Patients were treated with PSE.
    RESULTS: PSE produced an increase in thrombocyte count, and chemotherapy could be resumed.
    CONCLUSIONS: PSE seems to be a useful treatment for thrombocytopenia with splenomegaly associated with advanced gastric cancer and may allow continuation of chemotherapy.
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