OBJECTIVE: To delineate the natural history of splenic complications other than loss of splenic function in children with sickle cell disease (SCD), we performed a retrospective chart review of patients with SCD treated at the Texas Children\'s Hospital.
METHODS: We determined the dates of diagnoses of splenic complications, the number of acute splenic sequestration crises (ASSC), and hydroxyurea treatment in pediatric patients with SCD. We also examined the association of hydroxyurea therapy with the onset and severity of ASSC.
RESULTS: The cumulative prevalence of splenic complications was 24.7% for splenomegaly, 24.2% for ASSC, 9.6% for hypersplenism, and 5.6% for splenectomy. The cumulative prevalence of splenic complications was highest in patients with hemoglobin Sβ0 (69.2%), intermediate in hemoglobin SS (33.3%), low in hemoglobin SC (9.0%), and non-existent in hemoglobin Sβ+. The overall event rate of ASSC was 8.3 per 100 patient-years. The event-rate was 28.4 for hemoglobin Sβ0, 10.9 for hemoglobin SS, and 3.5 for hemoglobin SC. Patients with hemoglobin SS and hemoglobin Sβ0 on hydroxyurea therapy had a significantly higher occurrence of ASSC than those who were not, with event rates of 14.2 and 3.1, respectively. The event rate was also higher for children who started hydroxyurea before age 2 years than for those who started after this age (19.8 and 9.2, respectively).
CONCLUSIONS: The prevalence and severity of splenic problems vary widely between different sickle cell genotypes, with hemoglobin Sβ0 having the most severe complications. Hydroxyurea therapy is associated with increased incidence of ASSC, particularly when initiated before 2 years of age.

Introduction: Spleen angiosarcoma (SA) is a rare malignant neoplasm that arises from the splenic vascular endothelium, with only around 300 cases reported to date. Due to a limited number of reported cases, there is a paucity of data and a lack of understanding of its presentation, diagnosis, and management. In this study, we aim to provide a comprehensive review of SA.Areas covered: On 27 February 2021, a literature search was done in PubMed and Embase database. The search yielded 122 articles involving 205 patients. The focus was on patient demographics, risk factors, clinical presentations, investigation results, preliminary diagnoses, therapies provided, and patient outcomes. These factors were analyzed to identify possible risk factors, diagnostic modalities, and therapeutic principles that were not mentioned before.Expert opinion: The clinical presentation or investigation results of patients with SA are often nonspecific. Hence, they may not be sufficient to clinch the diagnosis of SA if used alone. The authors recommend a triple assessment of clinical examination, imaging findings, and pathology to diagnose SA with high accuracy. Splenectomy should be the mainstay of management, with chemotherapy and radiotherapy considered as adjuncts, especially in the presence of metastases.

UNASSIGNED: Whether hepatocellular carcinoma (HCC) patients with hypersplenism can benefit from splenectomy is unclear. This study aimed at exploring the efficacy and safety of concurrent splenectomy for HCC patients with hypersplenism.
UNASSIGNED: PubMed, EMBASE and Web of Science databases were systematically searched to compare data on the combination of hepatectomy or transhepatic arterial infusion (TAI) with splenectomy (the splenectomy group) with data on hepatectomy or TAI alone (the non-splenectomy group) for the treatment of HCC with hypersplenism. Prospective clinical trials or retrospective cohort studies from inception to May 10, 2020 were considered eligible for this analysis. The relevant outcomes, including patients\' demographics, clinicopathologic characteristics, perioperative indices and long-term outcomes, were independently extracted by two investigators. Publication bias for overall survival (OS) and disease-free survival (DFS) was qualitatively assessed by funnel plots and quantitatively evaluated by Begg\'s and Egger\'s tests.
UNASSIGNED: Nine retrospective studies including 1,650 patients were analyzed. Short-term outcomes suggested that the incidence rate of postoperative complications, including portal or splenic vein thrombosis [odds ratio (OR) =26.28, P<0.001] and pancreatic injury (OR =14.89, P=0.001), was significantly higher in the splenectomy group, whereas the perioperative mortality rate was similar between the splenectomy and non-splenectomy groups (P=0.541). Long-term outcomes indicated that the occurrence of variceal re-hemorrhage (OR =0.31, P<0.001) and tumor progress or recurrence (OR =0.62, P=0.001) was markedly reduced for patients who underwent splenectomy, while the long-term mortality rates were not statistically different between the two groups (P=0.087). The prognostic evaluation revealed that the OS and DFS were comparable between the splenectomy and non-splenectomy groups [for OS: hazard ratio (HR) =0.77, 95% confidence interval (CI): 0.53-1.13; for DFS: HR =0.87, 95% CI: 0.63-1.19]. Funnel plots suggested an HRs symmetric distribution for OS and DFS. Begg\'s and Egger\'s tests confirmed that there was no significant HR publication bias for OS and DFS.
UNASSIGNED: Due to the significant progress in surgical techniques and perioperative care, concomitant splenectomy should be considered as an optional treatment for some HCC patients with hypersplenism.

The majority of the global population of sickle cell disease (SCD) patients resides in Africa. Individuals with this condition are at great risk of serious infections and early mortality secondary to splenic dysfunction without preventative measures. This review investigated the spectrum of splenic complications encountered in SCD among populations in Africa. We systematically searched several databases for all articles published through March 3, 2020. We included 55 studies from 14 African countries. This review reveals the difference in frequency of splenic complications in SCD in Africa when compared with their counterparts in the United State and Europe. While several studies (n = 45) described splenomegaly with a prevalence of 12% to 73% among children, and 4% to 50% among adults with HbSS, the reported prevalence for acute splenic sequestration crisis (n = 6 studies) and hypersplenism (n = 4 studies) was <10% and <5% respectively. A total of 30 surgical splenectomy was reported across eight studies. Only two (3.7%) studies provided data on spleen function. A conflicting pattern was observed amongst studies that evaluated the relationship between splenomegaly and the presence of bacterial and malaria infections. This review reveals the paucity of studies describing the role of SCD-induced splenic dysfunction in morbidity and infection related mortality in Africa.

A large proportion of patients with chronic hepatitis C have associated thrombocytopenia (TCP). Due to bleeding risks, TCP, when severe, can limit diagnostic and therapeutic procedures, treatments, and increases risk of complications, especially excessive bleeding. It is important to understand the mechanisms that cause TCP in order to manage it. In general, TCP can be due to increased destruction or decreased production. Proposed mechanisms of increased destruction include autoantibodies to platelets and hypersplenism with sequestration. Proposed mechanisms of decreased production include virus-induced bone marrow suppression and decreased TPO production. Autoantibodies directed against platelet surface antigens have demonstrated an inverse correlation with platelet counts. Hypersplenism with sequestration involves the interaction of portal hypertension, splenomegaly, and platelet destruction. Decreased production mechanisms involve appropriate and inappropriate levels of TPO secretion. There is limited evidence to support viral-induced bone marrow suppression. In contrast, there is strong evidence to support low levels of TPO in liver failure as a major cause of TCP. TPO-agonists, specifically eltrombopag, have been shown in hepatitis C patients to increase platelet counts without reducing portal hypertension or splenomegaly. We conclude that TCP in hepatitis C virus-induced liver disease is often multifactorial, but an understanding of the mechanisms can lead to judicious use of new drugs for treatment.

Partial splenic embolization is a common procedure that reduces thrombocytopenia in patients with hypersplenism. The present review evaluated the adverse event profile of partial splenic embolization detailed in 30 articles. Although the technical success rate of the procedure in these papers is high, many patients experienced postprocedural complications. Minor complications such as postembolization syndrome occurred frequently. Major complications were less frequent but sometimes resulted in mortality. Underlying liver dysfunction and high infarction rates may be risk factors leading to major complications. Interventional radiologists should be aware of the complication profile of this procedure and further advance research in techniques dealing with hypersplenism.

 This review aimed to evaluate the safety, feasibility, and effectiveness of synchronous hepatectomy and splenectomy for selected patients with hepatocellular carcinoma and clinically significant portal hypertension compared to hepatectomy alone. A systematic search in electronic databases was conducted. A total of 8 studies including 1445 patients were reviewed. While perioperative safety was satisfactory, synchronous hepatectomy and splenectomy were associated with a significant improvement of 5-year overall survival (odds ratio = 1.81; 95% confidence interval: 1.35, 2.42; P < 0.0001).

It is well known that splenectomy is the standard of care in the management of clinically significant hypersplenism; however, some patients are found to be unacceptably high risk to tolerate open or even laparoscopic surgery. We present a 62-year-old female with significant comorbidities who was declared a very high risk for any open surgical intervention. She underwent splenic artery embolization with remarkable improvement of her platelet count. Her postoperative course was uneventful and the patient was discharged from the hospital on the fifth hospital day.

BACKGROUND: Idiopathic portal hypertension (IPH) is a disorder of unknown etiology and is characterized clinically by portal hypertension, splenomegaly, and hypersplenism accompanied by pancytopenia. This study evaluates the pathogenic concept of the disease by a systematic review of the literature and illustrates novel pathologic and laboratory findings.
METHODS: We report the first case of uncontrolled splenic hyperperfusion and enlargement with subsequent hypersplenism leading to life-threatening complications of IPH in infancy and emergent splenectomy.
CONCLUSIONS: Our results suggest that splenic NO and VCAM-1, rather than ET-1, have a significant impact on the development of IPH, even at a very early stage of disease. The success of surgical interventions targeting the splenic hyperperfusion suggests that the primary defect in the regulation of splenic blood flow seems to be crucial for the development of IPH. Thus, beside other treatment options splenectomy needs to be considered as a prime therapeutic option for IPH.

暂无摘要。