Patients with thrombosis at an unusual site will need to be explored for rare causes of thrombosis. Two of these rare causes include myeloproliferative neoplasms (MPNs) and paroxysmal nocturnal hemoglobinuria (PNH). It is important not to overlook these causes, since they require specific management, in addition to antithrombotic treatment (anticoagulants, antiplatelet agents). Unusual sites of venous thrombosis include upper extremity veins, splanchnic veins, cerebral veins, and retinal veins, and unusual sites of arterial thrombosis include renal, adrenal, splenic and mesenteric arteries, and intracardiac and aortal locations. Suspicion for MPN and PNH should be raised if there are concomitant abnormalities, such as elevated or decreased blood cell counts or splenomegaly. Diagnosis of MPN and PNH should include JAK2V617F mutational screening as well as flow cytometric assessment of GPI-anchored proteins in the peripheral blood, respectively. Specific treatments for MPN may include phlebotomy or cytoreductive drugs such as hydroxyurea, anagrelide, pegylated interferon-alpha, or Janus kinase inhibitors. Drugs used for PNH treatment include terminal complement inhibitors, such as eculizumab and ravulizumab, as well as proximally acting inhibitors such as pegcetacoplan or iptacopan. Patients with MPN and PNH are at high risk for thrombosis during their entire lifetime and should thus be followed by specialists experienced in the care of these diseases.

The impact of TIPS on waitlist mortality and liver transplantation (LT) urgency in patients with Budd-Chiari syndrome (BCS) remains unclear. We analyzed patients with BCS listed for LT in the UNOS database (2002-2024) to assess TIPS's impact on waitlist mortality and LT access through competing-risk analysis. We compared trends across 2 phases: phase 1 (2002-2011) and phase 2 (2012-2024). Of 815 patients with BCS, 263 (32.3%) received TIPS at listing. TIPS group had lower MELD-Na scores (20 vs. 22, p < 0.01), milder ascites ( p = 0.01), and fewer Status 1 patients (those at risk of imminent death while awaiting LT) (2.7% vs. 8.3%, p < 0.01) at listing compared to those without TIPS. TIPS patients had lower LT rates (43.3% vs. 56.5%, p < 0.01) and longer waitlist times (350 vs. 113 d, p < 0.01). TIPS use increased in phase 2 (64.3% vs. 35.7%, p < 0.01). Of 426 patients who underwent transplantation, 134 (31.5%) received TIPS, showing lower MELD-Na scores (24 vs. 27, p < 0.01) and better medical conditions (intensive care unit: 14.9% vs. 21.9%, p < 0.01) at LT. Status 1 patients were fewer (3.7% vs. 12.3%, p < 0.01), with longer waiting days (97 vs. 26 d, p < 0.01) in the TIPS group. TIPS use at listing increased from phase 1 (25.6%) to phase 2 (37.7%). From phase 1 to phase 2, ascites severity improved, re-LT cases decreased (phase 1: 9.8% vs. phase 2: 2.2%, p < 0.01), and cold ischemic time slightly decreased (phase 1: 7.0 vs. phase 2: 6.4 h, p = 0.14). Median donor body mass index significantly increased. No significant differences were identified in patient/graft survival at 1-/5-/10-year intervals between phases or TIPS/non-TIPS patients. While 90-day waitlist mortality showed no significant difference ( p = 0.11), TIPS trended toward lower mortality (subhazard ratio [sHR]: 0.70 [0.45-1.08]). Multivariable analysis indicated that TIPS was a significant factor in decreasing mortality (sHR: 0.45 [0.27-0.77], p < 0.01). TIPS group also showed significantly lower LT access (sHR: 0.65 [0.53-0.81], p < 0.01). Multivariable analysis showed that TIPS was a significant factor in decreasing access to LT (sHR: 0.60 [0.46-0.77], p < 0.01). Subgroup analysis excluding Status 1 or HCC showed similar trends. TIPS in patients with BCS listed for LT reduces waitlist mortality and LT access, supporting its bridging role.

Data detailing the risk of Venous Thromboembolism (VTE) subtypes among individuals with Inflammatory bowel disease (IBD) remain limited.Aims: We looked to assess the odds of VTE subtypes among hospitalized individuals with IBD as compared to those without IBD.

Using the Nationwide Inpatient Sample database, we applied a multivariable regression analysis to compare the odds of primary VTE-related hospitalizations among individuals with and without IBD from 2016 to 2020, including deep venous thrombosis (DVT), pulmonary embolism (PE), portal vein thrombosis (PVT), Budd Chiari syndrome (BCS), renal vein thrombosis (RVT), and cerebral venous sinus thrombosis (CVST).

Overall, there were 15,565 primary VTE-related hospitalizations among individuals with IBD, as compared to 1,449,164 among individuals without IBD. Having IBD increased the odds for DVT (aOR = 1.34, 95%CI: 1.25-1.43), PVT (aOR = 3.16, 95%CI: 2.65-3.76), and CVST (aOR=1.45, 95%CI: 1.05-2.00), without significant increase in the odds of a PE, BCS, or RVT. Further, individuals with ulcerative colitis (UC) were at a higher risk for the majority of VTE-subtypes as compared to those with Crohn's disease (CD). Among individuals with a VTE-related hospitalization, the presence of IBD was not associated with increased mortality (aOR = 0.77, 95%CI: 0.40-1.50), but was associated with an increased length of stay (CD - 4.8 days, UC - 5.3 days, without IBD - 4.3 days, p<0.01).

Clinicians should retain a high index of suspicion when evaluating VTE-related symptoms among individuals with IBD, as the presence of IBD confers a higher risk of DVT, PVT and CVST related-hospitalizations, and longer stays as compared to individuals without IBD.

Aortoesophageal fistula is rare and typically presents itself to the emergency department as Chiari's Triad of mid-thoracic pain, sentinel arterial hemorrhage, and exsanguination after a symptom-free interval. However, fatal bleeding may be the first and last presentation of an aortoesophageal fistula. When a patient experiences massive hematemesis without witnesses, EMS may assume that bleed is of a traumatic mechanism. We present a case of a 59-year-old male with no previous medical history who was transported to a trauma center unconscious and with massive bleeding of unknown origin. Computed tomography revealed a thoracic aortic aneurysm and an aortoesophageal fistula. Bleeding was not controlled and the patient expired. Trauma bay personnel should follow an algorithm which includes a prompt tamponade of the bleed using a Sengstaken-Blakemore tube or esophageal balloon paralleled by massive transfusion and obtaining an early computed tomography scan to manage patients with massive gastroesophageal bleeding until appropriate surgical interventions can be initiated.

To investigate the value of multiparametric MR imaging to differentiate between small hepatocellular carcinoma (s-HCC) versus benign liver lesions in patients with Budd-Chiari syndrome.

12 patients with benign hepatocellular lesions and 32 patients with small (<3 cm) HCCs were assessed. MRI images were reviewed by two radiologists blinded to the patient background information; lesion T1 and T2 signal intensities and ADC values were compared with the background liver. Enhancement of lesion relative to hepatic parenchyma [(T1Enh-T1liver)/T1liver] in the arterial, venous, and delayed phases was also compared between the two groups. A multivariable logistic model was developed using these categorical measures; the predictive value of the model was tested using the Area Under the Receiver operating characteristic (AU-ROC) curve for logistic models. P-values <0.05 were considered statistically significant.

There were consistent differences in T1lesion/T1liver, and T2lesion/T2liver, and ADClesion/ADCliver between benign hepatocellular lesions versus the sHCC group (p<0.001, p<0.001, p = 0.045, respectively). Lesion-to-background liver enhancement in the portal venous and delayed phases was different between the benign lesions versus sHCC (p=0.001). ROC analysis for the logistic model that included the T1 ratio, T2 ratio, and portal venous enhancement ratio demonstrated excellent discriminatory power with the area under the curve of 0.94.

Multiparametric MR imaging is a useful method to help differentiate benign liver lesions from sHCC in patients with Budd-Chiari syndrome.

Vascular, autoimmune hepatitis, and malignant causes of acute-on-chronic liver failure are rare but important to consider and investigate in patients with underlying liver disease who present with acute deterioration and other more common etiologies have been excluded. Vascular processes including Budd-Chiari syndrome and portal vein thrombosis require imaging for diagnosis and anticoagulation is the mainstay of therapy. Patients may require advanced interventional therapy including transjugular intrahepatic portosystemic shunt or consideration of liver transplantation. Autoimmune hepatitis is a complex disease entity that requires a high degree of clinical suspicion and can present heterogeneously.

Budd-Chiari syndrome (BCS) is a rare and potentially life-threatening disorder characterized by obstruction of the hepatic outflow tract. It is unknown whether patients with BCS represent a high risk for severe disease and mortality from coronavirus disease 2019 (COVID-19). Thus, we aimed to assess hospitalization rates, severe disease, all-cause mortality, intensive care unit (ICU) requirement and acute kidney injury (AKI) from COVID-19 diagnoses.

We identified 467 patients with BCS with COVID-19, 96 427 non-chronic liver disease (CLD) and 9652 non-BCS CLD. The BCS and non-CLD cohorts (n = 467 each) and BCS and non-BCS CLD (n = 440 each) were well balanced after propensity matching. When compared to the non-CLD cohort, the BCS group had a higher risk of all-cause mortality (5.1% vs. 2.4%, HR 2.18; 95% CI, 1.08-4.40), severe disease (6.0% vs. 2.4%, HR 2.20; 95% CI, 1.09-4.43), hospitalization (24.6% vs. 13.1%, HR 1.77; 95% CI, 1.30-2.42) and AKI (7.9% vs. 2.8%, HR 2.57; 95% CI, 1.37-4.85), but no significant differences in ICU requirements (2.4% vs. 2.1%, HR 0.75; 95% CI, 0.27-2.08) at 60-days time points. When compared to the non-BCS CLD cohort, the BCS group had a higher risk of all-cause mortality (3.6% vs. 2.5%, HR 3.94; 95% CI, 1.31-11.79), hospitalization (29.8% vs. 21.6%, HR 1.43; 95% CI, 1.09-1.86), but differences in ICU requirements (HR 0.90 (0.38-2.12)), AKI (HR 1.41 (0.86-2.30)) or severe disease (HR 1.92 (0.99-3.71)) did not reach statistical significance at 60-day follow up.

In conclusion, COVID-19 infection in patients with BCS is associated with poor outcomes. Patients with BCS infected with COVID-19 carry a significantly higher risk of hospitalization and all-cause mortality and a possible effect on severe disease and AKI compared with COVID-19 patients without CLD or with non-BCS-CLD.

Myeloproliferative neoplasms (MPNs) are defined as clonal disorders of the hematopoietic stem cell in which an exaggerated production of terminally differentiated myeloid cells occurs. Classical, Philadelphia-negative MPNs, i.e., polycythemia vera, essential thrombocythemia and primary myelofibrosis, exhibit a propensity towards the development of thrombotic complications that can occur in unusual sites, e.g., portal, splanchnic or hepatic veins, the placenta or cerebral sinuses. The pathogenesis of thrombotic events in MPNs is complex and requires an intricate mechanism involving endothelial injury, stasis, elevated leukocyte adhesion, integrins, neutrophil extracellular traps, somatic mutations (e.g., the V617F point mutation in the JAK2 gene), microparticles, circulating endothelial cells, and other factors, to name a few. Herein, we review the available data on Budd-Chiari syndrome in Philadelphia-negative MPNs, with a particular focus on its epidemiology, pathogenesis, histopathology, risk factors, classification, clinical presentation, diagnosis, and management.

Vascular liver diseases are an heterogenous group of diseases that collectively represent an important health issue in the field of liver diseases. This narrative review was elaborated by the Special Interest Group (SIG) "Gender in Hepatology" of the Italian Association for the Study of the Liver (AISF). We aimed to review the current knowledge regarding the potential role of biological sex in patients with vascular liver diseases such as splanchnic vein thrombosis, hepatic vein thrombosis, porto-sinusoidal vascular disorder, and hereditary hemorrhagic telangiectasia. As vascular liver diseases commonly affect young individuals, including women in childbearing age, we also included a specific section on the management of pregnancy in these challenging patients.

To evaluate clinical characteristics and factors associated with survival among liver transplantation (LT) recipients with Budd-Chiari syndrome (BCS), with or without transjugular intrahepatic portosystemic shunt (TIPS), in the post-Model for End-stage Liver Disease era.

We extracted data from the United Network for Organ Sharing database on all adult (≥18 y old) waitlisted candidates and recipients of LT with BCS in the United States between 2002 and 2019. Multivariable Cox regression was used to determine predictors of mortality and hazard ratios (HRs).

A total of 647 BCS patients were waitlisted between 2002 and 2019. BCS was an indication for LT in 378 (0.2%) of all adult LT recipients during the study period. Of BCS patients who received LT, approximately three-fourths (72.3%) were alive for up to 10 y. We found no significant difference in LT outcomes in BCS patients with or without TIPS. Longer length of hospital stay following LT (HR, 1.32; 95% confidence interval [CI], 1.19-1.47), Black/African American race (HR, 2.24; 95% CI, 1.38-3.64), diabetes (HR, 3.17; 95% CI, 1.62-6.21), donor risk index (HR, 1.44; 95% CI, 1.05-1.99), and lower albumin levels at the time of transplantation (HR, 0.66; 95% CI, 0.50-0.88) were negatively associated with survival after LT. Interestingly, neither the Model for End-stage Liver Disease nor prior TIPS showed a significant association with survival after LT.

These findings demonstrate good comparable survival among TIPS versus no TIPS in LT recipients with BCS. The decision for TIPS versus LT should be individualized on a case-by-case basis.

There is a paucity of data on the outcome of liver transplantation (LT) in Budd-Chiari Syndrome (BCS) patients who are listed as status 1. The objective of our study was to determine patient or graft survival following LT in status 1 BCS patients. We utilized United Network for Organ Sharing (UNOS) database to identify all adult patients (> 18 years of age) listed as status 1 with a primary diagnosis of BCS in the United States from 1998 to 2018, and analyzed their outcomes and compared it to non-status 1 BCS patients. Four hundred and forty-six patients with BCS underwent LT between 1998 and 2018, and of these 55 (12.3%) were listed as status 1. There was no difference in long-term post-liver transplant or "intention-to-treat" survival from the time of listing to death or the last day of follow-up between status 1 and non-status 1 groups. Graft and patient survival at 5 years for status 1 patients were 75% and 82%, respectively. Cox regression analysis showed that patients listed as status 1 (aHR: 0.45, p < .02) were associated with a better survival. BCS patients listed as status 1 have excellent survival following emergency LT.