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Background: Optimal anticoagulation management in patients with myeloproliferative neoplasms (MPN) experiencing splanchnic vein thrombosis (SpVT) requires balancing risks of bleeding and recurrent thrombosis., Objectives: We conducted a systematic review and meta-analysis to assess the incidence of bleeding and thrombosis recurrence in patients with MPN-SpVT., Methods: We included retrospective or prospective studies in English with ≥10 adult patients with MPN-SpVT. Outcomes included recurrent venous thrombosis (SpVT and non-SpVT), arterial thrombosis, and major bleeding. Pooled rates per 100 patient years with 95% CIs were calculated by DerSimonian-Laird method using random-effects model., Results: Out of 4624 studies screened, 9 studies with a total of 443 patients were included in the meta-analysis with median follow-up of 3.5 years. In the 364 patients with MPN-SpVT treated with anticoagulation, pooled event rate for major bleeding was 2.8 (95% CI, 1.5-5.1; I 2  = 95%), for recurrent venous thrombosis was 1.4 (95% CI, 0.8-2.2; I 2  = 72%), and for arterial thrombosis was 1.4 (95% CI, 0.6-3.3; I 2  = 92%) per 100 patient years. Among 79 patients (n = 4 studies) who did not receive anticoagulation, pooled event rate for major bleeding was 3.2 (95% CI, 0.7-12.7; I 2  = 97%), for recurrent venous thrombosis 3.5 (95% CI, 1.8-6.4; I 2  = 88%), and for arterial thrombosis rate 1.6 (95% CI, 0.4-6.6; I 2  = 95%) per 100 patient years., Conclusion: Patients with MPN-SpVT treated with anticoagulation have significant risks for both major bleeding and thrombosis recurrence. Further studies are necessary to determine the optimal anticoagulation approach in patients with MPN-SpVT., Competing Interests: Declaration of competing interests P.C., K.B., G.M.G., T.C., A.N., M.M., N.R., G.S., S.A., M.L., and J.B. have no conflict of interest. B.S. received grant support from AstraZeneca and Eisai, speaker honoraria from Eisai, and travel support from AbbVie, AstraZeneca, Ipsen, and Gilead, all not related to this study. P.E.R. has received research funding from Terrafirma; acted as a consultant for Hemostod, Mursla, Genfit, Boehringer Ingelheim, and Abbelight; received speaker fees from Tillots Pharma and AbbVie; and is supported by the Fondation pour la Recherche Médicale (FRM EQU202303016287), “Institut National de la Santé et de la Recherche Médicale” (ATIP AVENIR), the “Agence Nationale pour la Recherche” (ANR-18-CE14-0006-01, RHU QUID-NASH, ANR-18-IDEX-0001, ANR-22-CE14-0002), the “Émergence, Ville de Paris,” the Fondation ARC, the European Union’s Horizon 2020 research and innovation program under grant agreement number 847949, and by France 2030 Recherche Hospitalo-Universitaire Liver-Track, all not related to this study. W.A. has received research support from Bayer and has participated in advisory boards for AstraZeneca, Bayer, BMS-Pfizer, Leo Pharma, Norgine, Sanofi, and Viatris, all not related to this study. J.I.Z. has received research funding from Incyte and Quercegen; consultancy services to Sanofi, CSL, and Parexel; and honoraria from/advisory board participation with Pfizer/Bristol Myers Squibb (BMS), all outside current work. R.P. has received consultancy services from Merck Research Laboratory-Consultant, all outside the current work., (Copyright © 2024 International Society on Thrombosis and Haemostasis. Published by Elsevier Inc. All rights reserved.)

Splanchnic vein thrombosis (SVT) is a rare type of venous thromboembolism occurring within the splanchnic venous system. Portal vein thrombosis is the most common presentation, while Budd-Chiari syndrome is the least common. Liver cirrhosis and abdominal solid cancer are the main local risk factors for SVT, whereas myeloproliferative neoplasms are the predominant systemic risk factors. Signs and symptoms of SVT are nonspecific and include abdominal pain, gastrointestinal bleeding, and ascites. Asymptomatic SVT is not uncommon, and the majority would be detected incidentally on routine abdominal imaging performed for the follow-up of liver diseases and tumors. The management of SVT aims to prevent thrombus progression, promote vessel recanalization, and prevent recurrent venous thromboembolism. Anticoagulation should be started early in order to increase the chances of vessel recanalization and reduce the risk of portal hypertension-related complications. Direct oral anticoagulants have been included in recent guidelines, as alternatives to vitamin K antagonists, after clinical stability has been reached; however, caution is required in patients with liver or kidney dysfunction. Treatment duration is based on the presence (or absence) and type (transient vs. permanent) of risk factors. This narrative review aims to summarize the latest evidence on SVT, with a particular focus on the anticoagulant treatment in special categories of patients (e.g., liver cirrhosis, solid cancer, myeloproliferative neoplasms, pancreatitis, incidentally detected SVT, Budd-Chiari syndrome, and chronic SVT)., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

Introduction: Little is known about the long-term outcome of cirrhotic patients with splanchnic vein thrombosis (SVT). This prospective cohort study aimed to describe the clinical presentation, bleeding incidence, thrombotic events, and mortality in patients with SVT associated with cirrhosis., Methods: Among 604 consecutive patients with SVT enrolled over 2 years, 149 had cirrhosis. Major bleeding, thrombotic events, and all-cause mortality were recorded during a 2-year follow-up. In a subgroup, the degree of recanalization with or without anticoagulation therapy, and the correlation between clinical events and liver disease severity were also investigated., Results: The most common thrombosis sites were the portal (88%) and mesenteric veins (34%). At presentation, 50% of patients were asymptomatic. Anticoagulation was administered to 92/149 patients for a median of 6.5 months. Vessel recanalization was documented in 47/98 patients with a radiological follow-up. Anticoagulation was associated with a 3.33-fold higher of recanalization rate, and a lower recurrent thrombosis rate, while patients with and without anticoagulation experienced a similar rate of major bleeding episodes. Mortality rates were 6.8 per 100 patient-years for patients with thrombosis completely or partially resolving during the follow-up, and 15.4 per 100 patient-years for those with stable or progressing thrombosis. An impact of SVT on survival was only apparent in patients with more advanced liver disease (Child-Pugh B-C)., Conclusions: Patients with SVT and cirrhosis have a substantial long-term risk of recurrent thrombotic events, which is reduced by anticoagulation therapy without any increase in bleeding risk. Anticoagulation can improve the likelihood of vessel recanalization, and is associated with a lower risk of death for decompensated patients.

Splanchnic vein thrombosis (SVT) and cerebral vein thrombosis (CVT) are uncommon manifestation of venous thromboembolism (VTE), occurring less frequently than deep vein thrombosis of the lower extremities and pulmonary embolism. SVT encompasses portal vein thrombosis, mesenteric vein thrombosis, splenic vein thrombosis and the Budd-Chiari syndrome. It is therefore a heterogeneous disease, with differences in clinical manifestations according to the site of thrombosis. CVT includes thrombosis of the cortical or deep cerebral veins and thrombosis of the major dural venous sinuses. Clinical presentation is variable, with a wide spectrum of signs and symptoms that can mimic other cerebral diseases. There are no clinical algorithms or specific laboratory tests that can guide in the identification of SVT and CVT; therefore, the diagnosis relies exclusively on imaging tests. Conventional angiography once was the gold standard for the diagnosis of SVT and CVT, but it is rarely used nowadays. Abdominal ultrasound (US), computed tomography (CT) and magnetic resonance (MR) with angiography are currently used for the diagnosis of SVT; while cerebral CT and MR with angiography are currently used for the diagnosis of CVT. These imaging tests have different sensitivities/specificities and different advantages/disadvantages that should be kept into consideration when choosing the appropriate imaging test based on the suspected site of thrombosis. This narrative review summarizes the clinical and diagnostic approach to SVT and CVT., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Importance: Little information is available on the long-term clinical outcome of patients with splanchnic vein thrombosis (SVT)., Objective: To assess the incidence rates of bleeding, thrombotic events, and mortality in a large international cohort of patients with SVT., Design, Setting, and Participants: A prospective cohort study was conducted beginning May 2, 2008, and completed January 30, 2014, at hospital-based centers specialized in the management of thromboembolic disorders; a 2-year follow-up period was completed January 30, 2014, and data analysis was conducted from July 1, 2014, to February 28, 2015. Participants included 604 consecutive patients with objectively diagnosed SVT; there were no exclusion critieria. Information was gathered on baseline characteristics, risk factors, and antithrombotic treatment. Clinical outcomes during the follow-up period were documented and reviewed by a central adjudication committee., Main Outcomes and Measures: Major bleeding, defined according to the International Society on Thrombosis and Hemostasis; bleeding requiring hospitalization; thrombotic events, including venous and arterial thrombosis; and all-cause mortality., Results: Of the 604 patients (median age, 54 years; 62.6% males), 21 (3.5%) did not complete follow-up. The most common risk factors for SVT were liver cirrhosis (167 of 600 patients [27.8%]) and solid cancer (136 of 600 [22.7%]); the most common sites of thrombosis were the portal vein (465 of 604 [77.0%]) and the mesenteric veins (266 of 604 [44.0%]). Anticoagulation was administered to 465 patients in the entire cohort (77.0%) with a mean duration of 13.9 months; 175 of the anticoagulant group (37.6%) received parenteral treatment only, and 290 patients (62.4%) were receiving vitamin K antagonists. The incidence rates (reported with 95% CIs) were 3.8 per 100 patient-years (2.7-5.2) for major bleeding, 7.3 per 100 patient-years (5.8-9.3) for thrombotic events, and 10.3 per 100 patient-years (8.5-12.5) for all-cause mortality. During anticoagulant treatment, these rates were 3.9 per 100 patient-years (2.6-6.0) for major bleeding and 5.6 per 100 patient-years (3.9-8.0) for thrombotic events. After treatment discontinuation, rates were 1.0 per 100 patient-years (0.3-4.2) and 10.5 per 100 patient-years (6.8-16.3), respectively. The highest rates of major bleeding and thrombotic events during the whole study period were observed in patients with cirrhosis (10.0 per 100 patient-years [6.6-15.1] and 11.3 per 100 patient-years [7.7-16.8], respectively); the lowest rates were in patients with SVT secondary to transient risk factors (0.5 per 100 patient-years [0.1-3.7] and 3.2 per 100 patient-years [1.4-7.0], respectively)., Conclusions and Relevance: Most patients with SVT have a substantial long-term risk of thrombotic events. In patients with cirrhosis, this risk must be balanced against a similarly high risk of major bleeding. Anticoagulant treatment appears to be safe and effective in most patients with SVT.

Treatment of splanchnic vein thrombosis (SVT) is a clinical challenge due to heterogeneity of clinical presentations, increased bleeding risk, and lack of evidences from clinical trials. We performed an international registry to describe current treatment strategies and factors associated with therapeutic decisions in a large prospective cohort of unselected SVT patients. A total of 613 patients were enrolled (mean age 53.1 years, standard deviation ± 14.8); 62.6% males; the majority (468 patients) had portal vein thrombosis. Most common risk factors included cirrhosis (27.8%), solid cancer (22.3%), and intra-abdominal inflammation/infection (11.7%); in 27.4% of patients, SVT was idiopathic. During the acute phase, 470 (76.7%) patients received anticoagulant drugs, 136 patients (22.2%) remained untreated. Incidental diagnosis, single vein thrombosis, gastrointestinal bleeding, thrombocytopenia, cancer, and cirrhosis were significantly associated with no anticoagulant treatment. Decision to start patients on vitamin K antagonists after an initial course of parenteral anticoagulation was significantly associated with younger age, symptomatic onset, multiple veins involvement, and unprovoked thrombosis. Although a nonnegligible proportion of SVT patients did not receive anticoagulant treatment, the majority received the same therapies recommended for patients with usual sites thrombosis, with some differences driven by the site of thrombosis and the pathogenesis of the disease., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)

Splanchnic vein thrombosis (SVT) is an unusual manifestation of venous thromboembolism which involves one or more abdominal veins (portal, splenic, mesenteric and supra-hepatic veins). SVT may be associated with different underlying disorders, either local (abdominal cancer, liver cirrhosis, intra-abdominal inflammation or surgery) or systemic (hormonal treatment, thrombophilic conditions). In the last decades, myeloproliferative neoplasm (MPN) emerged as the leading systemic cause of SVT. JAK2 mutation, even in the absence of known MPN, showed a strong association with the development of SVT, and SVT was suggested to be the first clinical manifestation of MPN. Recently, an association between SVT, in particular supra-hepatic vein thrombosis, and paroxysmal nocturnal hemoglobinuria has also been reported. SVT occurs with heterogeneous clinical presentations, ranging from incidentally detected events to extensive thrombosis associated with overt gastrointestinal bleeding, thus representing a clinical challenge for treatment decisions. In the absence of major contraindications, anticoagulant therapy is generally recommended for all patients presenting with acute symptomatic SVT, but there is no consensus about the use of anticoagulant drugs in chronic or incidentally detected SVT. High quality evidence on the acute and long-term management is substantially lacking and the risk to benefit-ratio of anticoagulant therapy in SVT still needs to be better assessed., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Simple Summary: Cancer is associated with a high risk of developing venous thromboembolism, which includes thrombosis in unusual areas such as the abdominal veins (splanchnic, ovarian and renal veins). These thromboses are often incidental findings in the workup of a cancer patient. Cancer is one of the major risk factors for splanchnic vein thrombosis, ovarian vein thrombosis and renal vein thrombosis. Cancer-associated abdominal vein thrombosis carries high mortality rates and high risk of recurrent thrombosis. The management of cancer-associated abdominal vein thrombosis follows the general guidelines for the management of venous thromboembolism. Cancer is associated with an increased risk of developing venous thromboembolism, due to its direct influence on the three pillars of Virchow's triad (e.g., compression on the blood vessels by the tumour, blood vessels invasion, and cytokine release), together with the effect of exogenous factors (such as chemotherapy, radiotherapy, surgery). In cancer patients, the risk of thrombosis at unusual sites, such as splanchnic, ovarian and renal vein thrombosis, is also increased. Abdominal vein thromboses are frequently incidental findings on abdominal imaging performed as part of the diagnostic/staging workup or the follow-up care of malignancies. There is little evidence on the management of unusual site venous thromboembolism in cancer patients since there are only a few specific recommendations; thus, the management follows the general principles of the treatment of cancer-associated deep vein thrombosis and pulmonary embolism. This narrative review summarises the latest evidence on cancer-associated abdominal vein thrombosis, i.e., thrombosis of the splanchnic, ovarian and renal veins. [ABSTRACT FROM AUTHOR]

D-dimer is included in the diagnostic algorithm for deep vein thrombosis and pulmonary embolism. However, its role in the diagnosis of splanchnic vein thrombosis (SVT) is still controversial. The aim of this study was to evaluate the diagnostic accuracy of D-dimer for SVT. We performed a systematic review of the literature with meta-analysis (PROSPERO protocol registration number: CRD42020184300). The electronic databases MEDLINE, EMBASE, and CENTRAL were searched from inception to March 2021 week 4. Studies which evaluated D-dimer accuracy for SVT in any category of patients were selected. The index test was any D-dimer assay; the reference standard was any radiological imaging. The QUADAS-2 checklist was used for the risk of bias assessment. A bivariate random-effects regression model was used to calculate summary estimates of sensitivity and specificity. 12 studies (with a total of 1298 patients) evaluating the accuracy of D-dimer in patients at high risk of SVT (surgical patients, patients with liver cirrhosis or hepatocellular carcinoma) were included. None of the included studies was at low risk of bias. The weighted mean prevalence of SVT was 33.4% (95% CI, 22.5–45.2%, I2 = 94.8%). D-dimer accuracy was expressed by sensitivity 96% (95% CI, 72–100%); specificity 25% (95% CI, 5–67%); positive likelihood ratio 1.3 (95% CI, 0.9–1.9); negative likelihood ratio 0.16 (95% CI, 0.03–0.84); area under the ROC curve 0.80 (95% CI, 0.76–0.83). D-dimer seems to have high sensitivity in the diagnosis of patients at high-risk for SVT. However, there is a strong need for more robust evidence on this topic. [Display omitted] • D-dimer role in the diagnosis of splanchnic vein thrombosis (SVT) is controversial. • The diagnostic accuracy of D-dimer for SVT was evaluated in a systematic review. • D-dimer showed high sensitivity in patients at high-risk for SVT. • There was high risk of bias and high heterogeneity in the published literature. [ABSTRACT FROM AUTHOR]