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

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

OBJECTIVE: To determine the safety and usefulness of a two-tiered approach to balloon-occluded retrograde transvenous obliteration (B-RTO) as a treatment for large gastric varices after portal hypertension. MATERIALS AND METHODS: 50 patients were studied who underwent B-RTO for gastric varices between October 2004 and October 2011 in our institution. The B-RTO procedure was performed from the right femoral vein and the B-RTO catheter was retained until the following morning. Distribution of sclerotic agents in the gastric varices on fluoroscopy was evaluated in all patients on days 1 and 2. When distribution of sclerotic agents in the gastric varices on day 1 had been none or very scanty even though the volume of the sclerotic agent infused was above the acceptable level, a second infusion was administered on day 2. When distribution was satisfactory, the B-RTO catheter was removed. RESULTS: In 8 (16%) patients, little or no sclerotic agent infused on day 1 was distributed in the gastric varices. However, on day 2, sclerotic agents were distributed in all gastric varices. Mean volume of ethanolamine oleate-iopamidol infused on day 1 was 24.6 mL and was 19.4 mL on day 2. Gastric varices were well obliterated with no recurrence. Complications caused by the sclerotic agent such as pulmonary edema or renal insufficiencies were not seen. CONCLUSION: When gastric varices are very large, a strategy involving thrombosis of only the drainage vein on the first day followed by infusing the sclerotic agent on the following day might be effective and feasible.
Adult ; Aged ; Aged, 80 and over ; Balloon Occlusion/*methods ; Catheters, Indwelling ; Collateral Circulation ; Drug Administration Schedule ; Esophageal and Gastric Varices/etiology/radiography/*therapy ; Female ; Femoral Vein ; Gastrointestinal Hemorrhage/etiology/*therapy ; Humans ; Hypertension, Portal/*complications ; Iopamidol/*administration & dosage/adverse effects ; Male ; Middle Aged ; Oleic Acids/*administration & dosage/adverse effects ; Recurrence ; Retrospective Studies ; Sclerosing Solutions/*administration & dosage/adverse effects ; Tomography, X-Ray Computed

Female ; Humans ; Hypertension, Portal ; drug therapy ; etiology ; Liver Cirrhosis ; complications ; drug therapy ; Male ; Tetrazoles ; therapeutic use ; Valine ; analogs & derivatives ; therapeutic use ; Valsartan

Animals ; Colon ; blood supply ; pathology ; Colonic Diseases ; drug therapy ; etiology ; physiopathology ; Hemodynamics ; Hepatic Veins ; pathology ; physiopathology ; Hypertension, Portal ; complications ; physiopathology ; Intestinal Mucosa ; blood supply ; drug effects ; pathology ; Liver Cirrhosis, Experimental ; complications ; Losartan ; administration & dosage ; therapeutic use ; Male ; Microscopy ; Portal Pressure ; drug effects ; Random Allocation ; Rats ; Rats, Wistar

Ascites, hepatic encephalopathy and variceal hemorrhage are three major complications of portal hypertension. The diagnostic evaluation of ascites involves an assessment of its etiology by determining the serum-ascites albumin gradient and the exclusion of spontaneous bacterial peritonitis. Ascites is primarily related to an inability to excrete an adequate amount of sodium into urine, leading to a positive sodium balance. Sodium restriction and diuretic therapy are keys of ascites control. But, with the case of refractory ascites, large volume paracentesis and transjugular portosystemic shunts are required. In hepatorenal syndrome, splanchnic vasodilatation with reduction in effective arterial volume causes intense renal vasoconstriction. Splanchnic and/or peripheral vasoconstrictors with albumin infusion, and renal replacement therapy are only bridging therapy. Liver transplantation is the only definitive modality of improving the long term prognosis.
Anti-Bacterial Agents/therapeutic use ; Ascites/complications/*diagnosis/therapy ; Bacterial Infections/*diagnosis ; Hepatic Encephalopathy/complications ; Hepatorenal Syndrome/complications/*diagnosis/therapy ; Humans ; Hypertension, Portal/*complications ; Liver Transplantation ; Peritonitis/*diagnosis/drug therapy/etiology ; Serum Albumin/administration & dosage

Endoscopy, Digestive System ; Esophageal and Gastric Varices ; complications ; drug therapy ; surgery ; therapy ; Fibrosis ; complications ; Gastrointestinal Hemorrhage ; drug therapy ; etiology ; surgery ; therapy ; Humans ; Hypertension, Portal ; drug therapy ; etiology ; surgery ; therapy

Gastrointestinal Hemorrhage ; drug therapy ; etiology ; prevention & control ; Humans ; Hypertension, Portal ; complications

OBJECTIVE: To determine the effect of angiotensin II receptor 1 antagonist losartan on portal hypertensive gastropathy (PHG) in rats and its mechanism. METHODS: Forty-eight Sprague-Dawley rats were randomly divided into 4 groups: a sham-operated group, a model group, a treatment group, and a prevention group. The partial portal vein and left suprarenal vein of rats were ligated to develop PHG. Portal vein pressure (PVP), the level of angiotensin IIin blood, gastric injury index(GI), and pathological diagnosis integral(PI) were measured. In situ hybridization was used to determine the expression and immunolocalization of angiotensin II receptor 1 in rat stomach wall. RESULTS: PVP, GI, and PI of the treatment group and the prevention group were evidently reduced (P<0.01), and the level of angiotensin IIin blood increased obviously. The expression of angiotensin II receptor 1 was negative in the control group, increased significantly in the model group, and decreased significantly in the treatment group and the prevention group. CONCLUSION The expression of angiotensin II receptor 1 elevates in portal hypertensive gastropathy. Losartan can reduce PVP, inhibit the activation of angiotensin II receptor 1 in gastric submucous layer, and has therapeutic effect on PHG.
Angiotensin II ; blood ; Angiotensin II Type 1 Receptor Blockers ; pharmacology ; therapeutic use ; Animals ; Gastric Mucosa ; drug effects ; metabolism ; Hypertension, Portal ; blood ; complications ; drug therapy ; Losartan ; pharmacology ; therapeutic use ; Male ; RNA, Messenger ; genetics ; metabolism ; Random Allocation ; Rats ; Rats, Sprague-Dawley ; Receptor, Angiotensin, Type 1 ; genetics ; Stomach Diseases ; blood ; drug therapy ; etiology

Portal hypertension as a consequence of liver cirrhosis is responsible for serious complications such as variceal bleeding, ascites and hepatic encephalopathy. Successful pharmacological treatment of portal hypertension can prevent the risk of the variceal bleeding, and contribute to reduce the morbidity and mortality in patients with liver cirrhosis. To identify the effect of drugs on portal hypertension, portal pressure was evaluated accurately before and after the drug administration. The hepatic venous pressure gradient has been accepted as the gold-standard method for assessing the severity of portal hypertension and the response to drug treatment. The mean hepatic venous pressure gradient was 15.1+/-5.4 mmHg in Korean cirrhotic patients who had experienced variceal bleeding. Non-selective beta blockers are the treatment of choice for primary and secondary prevention of variceal bleeding. The dose of propranolol should be subsequently adjusted until the resting heart rate had been reduced by 25% or less than 55 beats per minute. It has been reported that the optimal dose of propranolol is variable due to racial differences in cardiovascular receptor sensitivity. In Korean patients with portal hypertension and liver cirrhosis, the mean required dose of propranolol to reach target heart rate was 165 mg (range; 80-280 mg). This review covers mainly the results of the pharmacological therapy of portal hypertension in Korean cirrhotic patients.
Adrenergic beta-Antagonists/administration & dosage ; Hepatic Veins ; Humans ; Hypertension, Portal/diagnosis/*drug therapy/physiopathology ; Korea ; Liver Cirrhosis/complications/physiopathology ; Propranolol/administration & dosage ; Venous Pressure/drug effects