Primary myelofibrosis (PMF) is easily confused with cirrhosis, due to its main clinical manifestations of splenomegaly and the blood cytopenia. This review focuses on clinical studies to identify primary myelofibrosis and cirrhosis related portal hypertension, to analyze the differences between the two diseases, in order to distinguish PMF and cirrhosis from the pathogenesis, clinical manifestations, laboratory examinations and treatment principles, and simultaneously improve clinicians' understanding of PMF, which is a reference for exploring the early screening or diagnostic indicators of PMF, also provides a clinical basis for the application of new targeted drugs such as ruxolitinib.
Humans ; Primary Myelofibrosis/drug therapy* ; Hypertension, Portal/complications* ; Liver Cirrhosis/pathology* ; Splenomegaly/pathology* ; Anemia

At present, there is no uniform standard for diagnosis and treatment of portal hypertension complicated with hepatocellular carcinoma internationally. Although in recent years, with the significant advances of surgical technique and the positive progress of targeted and immunotherapy in the field of hepatocellular carcinoma, the survival of hepatocellular carcinoma patients has improved, but the risk of surgery in patients with portal hypertension complicated with hepatocellular carcinoma remains high, and surgical treatment is still controversial. Therefore, based on the existing evidence, the Chinese Society of Spleen and Portal Hypertension Surgery, Chinese Society of Surgery, Chinese Medical Association has organized relevant experts to develop the consensus on clinical diagnosis and treatment of portal hypertension with hepatocellular carcinoma (2022) after full discussion. This consensus aims to provide the latest guidance for the standardized diagnosis and treatment of portal hypertension with hepatocellular carcinoma in China. Given that most portal hypertension originates from cirrhosis, this consensus only addresses the diagnosis and treatment of cirrhosis-related portal hypertension with hepatocellular carcinoma.
Carcinoma, Hepatocellular/therapy* ; Consensus ; Humans ; Hypertension, Portal/therapy* ; Liver Cirrhosis/complications* ; Liver Neoplasms/therapy*

The Baveno VII workshop held in October 2021 was featured by the subject of personalized care in portal hypertension. The workshop focused on the following 9 topics including: the relevance and indications for measuring the hepatic venous pressure gradient as a gold standard; the use of non-invasive tools for the diagnosis of compensated advanced chronic liver disease and clinically significant portal hypertension; the impact of etiological and of non-etiological therapies in the course of cirrhosis; the prevention of the first episode of decompensation; the management of the acute bleeding episode; the prevention of further decompensation; as well as the diagnosis and management of splanchnic vein thrombosis and other vascular disorders of the liver. This essay provides a compilation and summary of recommendations regarding the abovementioned topics, and presents the most recent research proceedings and the corresponding consensus to our readers.
Consensus ; Esophageal and Gastric Varices ; Humans ; Hypertension, Portal/therapy* ; Liver Cirrhosis/therapy* ; Portal Pressure

This article presented an overview of the therapeutic effects of Chinese medicine (CM) preparations for promoting blood circulation and removing blood stasis for patients with portal vein thrombosis (PVT) after splenectomy. Based on published clinical researches of CM preparations for PVT after splenectomy in patients with cirrhotic portal hypertension (CPH), this paper evaluated the incidence of PVT, and explored potential active components and mechanisms of CM preparations. Safflower Yellow Injection, Danshen Injection () Danhong Injection (), and Compound Danshen Dropping Pill () achieved good curative effect alone or combined with anticoagulant therapy. In addition, Compound Biejia Ruangan Tablet () and Anluo Huaxian Pill () can also significantly improve the hemodynamic disorders of portal vein system in patients with cirrhosis. Considering the role of CM preparations in ameliorating the incidence of PVT after splenectomy in patients with CPH, we suggested that future research should provide more attention to CM alone or CM combined with anticoagulant for cirrhosis with PVT.
Anticoagulants/therapeutic use* ; Humans ; Hypertension, Portal/drug therapy* ; Liver Cirrhosis/surgery* ; Medicine, Chinese Traditional/adverse effects* ; Portal Vein ; Risk Factors ; Splenectomy/adverse effects* ; Venous Thrombosis/etiology*

Portal hypertension(PH) is one of the main complications of cirrhosis.Transjugular intrahepatic portosystemic shunt(TIPS) is the percutaneous creation of a conduit from the hepatic vein to the portal vein that is used to manage consequences of PH (i.e., variceal bleeding and refractory ascites) and used as a bridging therapy to liver transplant for decompensated cirrhosis. The following Clinical Practice Guidelines (CPGs) presents profession associational recommendations of the Chinese College of Interventionalists(CCI) on TIPS for PH. The CPGs was written by more than 30 experts in the field of TIPS in China (including interventional radiologists, liver surgeons, hepatologists and gastroenterologist, et al.). The panel of experts, produced these CPGs using evidence from PubMed and Cochrane database searches and combined with relevant expert consensuses and high quality clinical researches in China providing up to date guidance on TIPS for PH with the only purpose of improving clinical practice.
China ; Esophageal and Gastric Varices/therapy* ; Gastrointestinal Hemorrhage/therapy* ; Humans ; Hypertension, Portal/therapy* ; Liver Cirrhosis/therapy* ; Portasystemic Shunt, Transjugular Intrahepatic ; Treatment Outcome

OBJECTIVE: To evaluate the feasibility, safety, and clinical outcomes of plug-assisted retrograde transvenous obliteration (PARTO) to treat gastric variceal hemorrhage in patients with portal hypertension. MATERIALS AND METHODS: From May 2012 to June 2014, 19 patients (11 men and 8 women, median age; 61, with history of gastric variceal hemorrhage; 17, active bleeding; 2) who underwent PARTO using a vascular plug and a gelfoam pledget were retrospectively analyzed. Clinical and laboratory data were examined to evaluate primary (technical and clinical success, complications) and secondary (worsening of esophageal varix [EV], change in liver function) end points. Median follow-up duration was 11 months, from 6.5 to 18 months. The Wilcoxon signed-rank test was used to compare laboratory data before and after the procedure. RESULTS: Technical success (complete occlusion of the efferent shunt and complete filling of gastric varix [GV] with a gelfoam slurry) was achieved in 18 of 19 (94.7%) patients. The embolic materials could not reach the GV in 1 patient who had endoscopic glue injection before our procedure. The clinical success rate (no recurrence of gastric variceal bleeding) was the same because the technically failed patient showed recurrent bleeding later. Acute complications included fever (n = 2), fever and hypotension (n = 2; one diagnosed adrenal insufficiency), and transient microscopic hematuria (n = 3). Ten patients underwent follow-up endoscopy; all exhibited GV improvement, except 2 without endoscopic change. Five patients exhibited aggravated EV, and 2 of them had a bleeding event. Laboratory findings were significantly improved after PARTO. CONCLUSION: PARTO is technically feasible, safe, and effective for gastric variceal hemorrhage in patients with portal hypertension.
Aged ; Balloon Occlusion ; Embolization, Therapeutic ; Endoscopy, Digestive System ; Esophageal and Gastric Varices/complications/radiography/*therapy ; Female ; Gastrointestinal Hemorrhage/therapy ; Gelatin Sponge, Absorbable/chemistry ; Humans ; Hypertension, Portal/complications ; Male ; Middle Aged ; Retrospective Studies ; Tomography, X-Ray Computed

Portal vein embolization (PVE) is known as an effective and safe preoperative procedure that increases the future liver remnant (FLR) in patients with insufficient FLR. However, some possible major complications can lead to non-resectability or delayed elective surgery that results in increased morbidity and mortality. Although the majority of these complications are rare, knowledge of the radiologic findings of post-procedural complications facilitate an accurate diagnosis and ensure prompt management. We accordingly reviewed the CT findings of the complications of PVE.
Aged ; Cholangiocarcinoma/radiography/therapy ; Embolization, Therapeutic/*adverse effects ; Female ; Humans ; Hypertension, Portal/etiology ; Liver Neoplasms/radiography/*therapy ; Male ; Middle Aged ; Portal Vein/*radiography ; Tomography, X-Ray Computed ; Vascular System Injuries/etiology ; Venous Thrombosis/etiology

The management of hepatocellular carcinoma (HCC) is decided according to the evidence base recommendations generated by international societies especially by Barcelona clinical liver cancer (BCLC) guideline. However, the BCLC guideline based on studies of the Western countries, has not been well matched to real life cohort in Korea. In Western countries, a deceased donor liver transplantation has been well allocated to the HCC patients with preserved liver function. Patients with mild to moderate portal hypertension and certain BCLC B patients could be eligible for hepatic resection if a chance for 50% survival rate at 5 years is perceived. If liver transplantation (LT) is back up for liver resection in those patients as a salvage therapy, widening indication of liver resection could be much easily acceptable. On the other hands, new selection criteria of HCC beyond Milan criteria considering tumor biology, has been provided in the field of LT resulting in more than 50% survival rate at 5 years. Herein, surgical perspectives beyond the BCLC recommendation for LT for HCC would be reviewed in the respect of Korean surgeon's view in this article.
Biology ; Carcinoma, Hepatocellular* ; Cohort Studies ; Hand ; Humans ; Hypertension, Portal ; Korea ; Liver Neoplasms* ; Liver Transplantation* ; Liver* ; Patient Selection ; Salvage Therapy ; Survival Rate ; Tissue Donors

BACKGROUND/AIMS: Angiotensin receptor blockers (ARBs) inhibit activated hepatic stellate cell contraction and are thought to reduce the dynamic portion of intrahepatic resistance. This study compared the effects of combined treatment using the ARB candesartan and propranolol versus propranolol monotherapy on portal pressure in patients with cirrhosis in a prospective, randomized controlled trial. METHODS: Between January 2008 and July 2009, 53 cirrhotic patients with clinically significant portal hypertension were randomized to receive either candesartan and propranolol combination therapy (26 patients) or propranolol monotherapy (27 patients). Before and 3 months after the administration of the planned medication, the hepatic venous pressure gradient (HVPG) was assessed in both groups. The dose of propranolol was subsequently increased from 20 mg bid until the target heart rate was reached, and the candesartan dose was fixed at 8 mg qd. The primary endpoint was the HVPG response rate; patients with an HVPG reduction of >20% of the baseline value or to <12 mmHg were defined as responders. RESULTS: The mean portal pressure declined significantly in both groups, from 16 mmHg (range, 12-28 mmHg) to 13.5 mmHg (range, 6-20 mmHg) in the combination group (P<0.05), and from 17 mmHg (range, 12-27 mmHg) to 14 mmHg (range, 7-25 mmHg) in the propranolol monotherapy group (P<0.05). However, the medication-induced pressure reduction did not differ significantly between the two groups [3.5 mmHg (range, -3-11 mmHg) vs. 3 mmHg (range, -8-10 mmHg), P=0.674]. The response rate (55.6% vs. 61.5%, P=0.435) and the reductions in mean blood pressure or heart rate also did not differ significantly between the combination and monotherapy groups. CONCLUSIONS: The addition of candesartan (an ARB) to propranolol confers no benefit relative to classical propranolol monotherapy for the treatment of portal hypertension, and is thus not recommended.
Adolescent ; Adult ; Aged ; Antihypertensive Agents/*therapeutic use ; Benzimidazoles/*therapeutic use ; Blood Pressure ; Drug Therapy, Combination ; Female ; Humans ; Hypertension, Portal/complications/*drug therapy ; Liver Cirrhosis/complications/diagnosis ; Male ; Middle Aged ; Propranolol/*therapeutic use ; Prospective Studies ; Tetrazoles/*therapeutic use ; Treatment Outcome ; Young Adult

Embolization, Therapeutic ; Humans ; Hypertension, Portal ; therapy ; Portasystemic Shunt, Surgical