Hypersplenism

脾功能亢进
  • 文章类型: Journal Article
    部分脾栓塞术(PSE)联合经动脉化疗栓塞术(TACE)已被报道用于治疗肝硬化脾功能亢进和血小板减少症的肝细胞癌(HCC)。然而,需要时重复PSE的疗效和安全性尚不清楚。本研究旨在探讨外周血细胞和肝功能的术后变化,无进展生存期(PFS),与单独接受TACE相比,接受TACE和重复PSE的脾功能亢进HCC患者的安全性和安全性.
    这项回顾性研究纳入了102例于2014年1月至2021年12月接受TACE(n=73)或TACE+PSE(n=29)的脾功能亢进肝癌患者。在1周时调查外周血细胞和肝功能的变化,2、6、12、18和24个月。记录TACE程序和不良事件。分析PFS及预后因素。
    尽管对初始PSE的反应有限,反复PSE再次增加血小板(PLT),在18个月时达到顶峰。它还继续改善红细胞(RBC)和血红蛋白,这表明两组之间从基线到24个月的变化存在显着差异,以及12个月和18个月时的Child-Pugh评分。TACE+PSE组平均TACE疗程明显高于单纯TACE组(4.55vs3.26,P=0.019)。TACE+PSE组的中位PFS较长(19.4vs9.5个月,P=0.023)比单纯TACE组,其中PSE是一个独立的保护因素(HR,0.508;P=0.014)。初始和重复PSE在安全性方面没有显着差异。
    重复PSE对再次增加PLT和改善RBC有效,血红蛋白和肝功能。它有助于此后执行串行TACE程序。TACE合并反复PSE的PFS明显长于单独TACE,其中PSE是独立的保护因素。此外,重复PSE的安全性与初始PSE相当.
    UNASSIGNED: Partial splenic embolization (PSE) combined with transarterial chemoembolization (TACE) has been reported in treatment of hepatocellular carcinoma (HCC) with cirrhotic hypersplenism and thrombocytopenia. However, efficacy and safety of repeated PSE when required are unclear. This study aims to investigate post-procedural changes in peripheral blood cell and hepatic function, progression-free survival (PFS), and safety of HCC patients with hypersplenism received TACE and repeated PSE compared to those received TACE alone.
    UNASSIGNED: This retrospective study included 102 HCC patients with hypersplenism who received TACE (n = 73) or TACE+PSE (n = 29) from January 2014 to December 2021. Changes in peripheral blood cell and hepatic function were investigated at 1 week, 2, 6, 12, 18, and 24 months. TACE procedure sessions and adverse events were recorded. PFS and prognostic factors were analyzed.
    UNASSIGNED: Despite response to initial PSE being limited, repeated PSE increased platelet (PLT) again, which peaked at 18 months. It also continued to improve red blood cell (RBC) and hemoglobin, which showed significant differences in changes from baseline between two groups until 24 months, as well as Child-Pugh scores at 12 and 18 months. Mean TACE procedure sessions were significantly higher in TACE+PSE group than that in TACE alone group (4.55 vs 3.26, P = 0.019). TACE+PSE group had longer median PFS (19.4 vs 9.5 months, P = 0.023) than TACE alone group, where PSE was an independent protective factor (HR, 0.508; P = 0.014). Initial and repeated PSE showed no significant differences in safety.
    UNASSIGNED: Repeated PSE is effective in increasing PLT again and improving RBC, hemoglobin and liver function. It contributed to performing serial TACE procedures thereafter. TACE combined with repeated PSE has significantly longer PFS than TACE alone, where PSE was an independent protective factor. Moreover, the safety of repeated PSE was comparable to initial PSE.
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  • 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
    一名70多岁的男子尽管诊断为2型糖尿病,但仍有低糖化血红蛋白(HbA1c)值的病史。他的血糖读数在8到15mmol/L之间,但他的HbA1c值低于27mmol/mol。初步调查显示,红细胞寿命减少是导致HbA1c值低且具有误导性的原因。进一步调查显示慢性肝病和脾肿大,脾功能亢进可能是红细胞更新增加的原因。HbA1c估计不再可靠,因此,正在进行的糖尿病护理是在家庭毛细血管血糖监测的指导下进行的。医疗保健提供者和临床实验室人员需要意识到2型糖尿病患者中HbA1c值非常低的可能临床意义。
    A man in his 70s presented with a history of low glycated haemoglobin (HbA1c) values despite a diagnosis of type 2 diabetes. His blood glucose readings ranged between 8 and 15 mmol/L, but his HbA1c values were below 27 mmol/mol. Initial investigations demonstrated evidence of reduced red blood cell lifespan as a cause of misleadingly low HbA1c values. Further investigation revealed chronic liver disease and splenomegaly, with hypersplenism being the probable cause of increased red blood cell turnover. HbA1c estimation was no longer reliable, so ongoing diabetic care was guided by home capillary blood glucose monitoring. Healthcare providers and clinical laboratorians need to be aware of the possible clinical implications of very low HbA1c values in patients with type 2 diabetes.
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  • 文章类型: Journal Article
    背景:内脏利什曼病(VL),或者kala-azar,是流行地区艾滋病患者的常见共病。尽管有病毒学控制,许多患者仍继续经历VL复发,而是免疫失败.这些患者仍有慢性脾功能亢进症状,例如贫血,白细胞减少症,和血小板减少症,并且由于CD4+计数低而有严重合并感染的风险。因此,在这项研究中,对脾切除的VL和HIV感染患者进行了调查,以了解为什么这些患者的CD4+计数无法恢复,评估脾肿块对脾功能亢进和免疫衰竭的重要性。
    方法:从一个回顾性的开放队列中,对13例患者进行了脾切除术作为复发性VL的抢救治疗,调查了11例HIV感染患者。这项研究比较了脾切除术前后患者的全血细胞计数(CBC)以及CD4和CD8细胞计数与脾脏重量的关系。
    结果:脾切除术后CBC明显改善,表明脾功能亢进.然而,据我们所知,这是第一项研究表明脾脏质量与CD4细胞计数呈强烈负相关(ρ=-0.71,P=0.015)。
    结论:这一发现出乎意料,因为脾脏是最广泛的淋巴组织和T淋巴细胞来源。在回顾了文献和推理之后,我们假设免疫衰竭是继发于最初由生产性HIV感染诱导的脾细胞凋亡引起的CD4+损失,随后,通过脾巨噬细胞寄生虫感染维持的焦亡。
    BACKGROUND: Visceral leishmaniasis (VL), or kala-azar, is a common comorbidity in patients with AIDS in endemic areas. Many patients continue to experiences relapses of VL despite virological control, but with immunological failure. These patients remain chronically symptomatic with hypersplenism, for example with anemia, leukopenia, and thrombocytopenia, and are at risk of severe co-infection due to low CD4+ count. Therefore, in this study, splenectomized patients with VL and HIV infection were investigated to understand why the CD4+ count fails to recover in these patients, evaluating the importance of spleen mass for hypersplenism and immunological failure.
    METHODS: From a retrospective open cohort of 13 patients who had previously undergone splenectomy as salvage therapy for relapsing VL, 11 patients with HIV infection were investigated. This study compared the patients\' complete blood cell count (CBC) and CD4+ and CD8+ cell counts before and after splenectomy with respect to spleen weight.
    RESULTS: CBC was substantially improved after splenectomy, indicating hypersplenism. However, to the best of our knowledge, this is the first study to show that spleen mass is strongly and negatively correlated with CD4+ cell count (ρ = -0.71, P = 0.015).
    CONCLUSIONS: This finding was unexpected, as the spleen is the most extensive lymphoid tissue and T-lymphocyte source. After reviewing the literature and reasoning, we hypothesized that the immunological failure was secondary to CD4+ loss initially by apoptosis in the spleen induced by productive HIV infection and, subsequently, by pyroptosis sustained by parasitic infection in spleen macrophages.
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  • 文章类型: Case Reports
    一名31岁的Child\'sB肝硬化伴门静脉高压和脾肿大的妇女,有一个月的腹痛史。体格检查证实脾肿大。血液调查显示白细胞(WBC)和血小板计数较低。计算机断层扫描(CT)显示远端脾动脉处的脾动脉瘤,大小为3.4x3.4x4.3cm(APxWxCC),伴有血栓和脾梗塞。成功进行了血管造影栓塞,没有立即出现并发症。腹痛症状缓解,导致患者在栓塞后第三天从病房出院。外科门诊的随访显示病人仍无症状,重复的血细胞计数显示WBC和血小板计数均有所改善。此外,随访CT扫描显示脾脏大小减小,表明积极的结果和对干预的积极反应。
    A 31-year-old woman with Child\'s B liver cirrhosis with portal hypertension and splenomegaly presented with a one-month history of abdominal pain. A physical examination confirmed splenomegaly. A blood investigation revealed a low white blood cell (WBC) and platelet count. Computed tomography (CT) revealed a splenic artery aneurysm at the distal splenic artery measuring 3.4 x 3.4 x 4.3 cm (AP x W x CC) with thrombus and splenic infarction. A successful angiographic embolisation was performed without immediate complications. The abdominal pain symptoms resolved, leading to the patient\'s discharge from the ward on the third day post-embolisation. Follow-up at the surgical outpatient clinic indicated the patient remained asymptomatic, and repeated blood counts showed improvement in both WBC and platelet counts. Furthermore, follow-up CT scans demonstrated a reduction in spleen size, indicating positive outcomes and a favourable response to the intervention.
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    探讨肝硬化患者部分脾动脉栓塞(PSE)后门静脉血栓形成(PVT)的危险因素。
    作者回顾性分析了在2020年1月至2021年12月期间接受部分脾动脉栓塞治疗的151例肝硬化脾功能亢进患者。根据PSE后是否发生PVT,将患者分为PVT组和非PVT组。进行单因素分析以选择PSE后PVT的危险因素,多变量分析用于分析单变量分析中P值小于0.1的变量。
    有151名患者参加了这项研究,PVT组22例,非PVT组129例。在年龄方面没有显著差异,性别,吸烟,高血压,糖尿病,Child-Pugh在两组之间。PVT组和非PVT组PSE后的白细胞(WBC)和血小板计数均明显高于PSE前。单因素分析显示门静脉血流速度,PSE后食管静脉曲张结扎和WBC的P值小于0.1。多因素分析显示,门静脉血流速度是PSE后PVT的相关因素。
    门静脉血流速度是PSE后PVT的相关因素。在患者接受PSE之前,应考虑门静脉血流速度。
    UNASSIGNED: To investigate risk factors for portal venous thrombosis (PVT) after partial splenic artery embolization (PSE) in hepatic cirrhosis patients.
    UNASSIGNED: The authors retrospectively analyzed 151 hepatic cirrhosis patients with hypersplenism who underwent partial splenic artery embolization between January 2020 and December 2021. The patients were divided into a PVT group and a non-PVT group according to whether they had PVT after PSE. Univariate analyses were performed to select risk factors for PVT after PSE, and multivariate analysis was used to analyze variates with a value of P less than 0.1 in univariate analysis.
    UNASSIGNED: There were 151 patients enroled in the study, with 22 patients in the PVT group and 129 patients in the non-PVT group. There was no significant difference in terms of age, sex, smoking, hypertension, diabetes, Child-Pugh between two groups. White blood cell (WBC) and platelet counts after PSE were significantly higher than those before PSE in both the PVT group and non-PVT group. Univariate analysis showed that portal venous blood flow velocity, ligation of oesophageal varices and WBC after PSE were found to have a P value less than 0.1. Multivariate analysis showed that portal venous blood flow velocity was a factor associated with PVT after PSE.
    UNASSIGNED: Portal venous blood flow velocity was a factor associated with PVT after PSE. Portal venous blood flow velocity should be considered before patients undergo PSE.
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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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