Liver involvement is often observed in hematological disorders, resulting in liver abnormality, including unconjugated hyperbilirubinemia, monoclonal hyperglobulinemia, portal vein, or hepatic vein thrombosis or portal hypertension, hepatosplenomegaly, or iron accumulation in the liver. Here we summarize the major hematological diseases that often affect the liver: hemolytic anemia, defect in coagulation or anti-coagulation factors, myeloproliferative neoplasm, hemophagocytic lymphohistiocytosis, multiple myeloma, leukemia, and lymphoma. We hope this review will help clinicians diagnose and manage the patients with liver involvement by hematological disorders.
Hematologic Diseases ; Humans ; Hypertension, Portal ; Myeloproliferative Disorders/diagnosis* ; Portal Vein/pathology*

Chronic liver disease is a major disorder worldwide. A better understanding of anatomy, blood flow, and pathophysiology may be a key issue for their proper management. Ultrasound (US) is a simple and non-invasive diagnostic tool in the abdominal field. Doppler mode offers real-time hemodynamic evaluation, and the contrast-enhanced US is one of the most frequently used modalities for the detailed assessment. Further development in digital technology enables three-dimensional (3D) visualization of target images with high resolution. This article reviews the wide ranges of application in the abdominal US and describes the recent progress in the diagnosis of chronic liver diseases.
Diagnosis ; Fibrosis ; Hemodynamics ; Hypertension, Portal ; Liver Diseases ; Liver ; Ultrasonography

A 2-year-old, spayed female, Korean domestic short-hair cat was presented with depression and vomiting. The patient had history of weight loss lasting seven months. Physical examination revealed icterus in the pinna, oral mucosa, and sclera. Based on ultrasonography and computed tomography, tentative diagnosis was extrahepatic biliary tract obstruction with acquired portosystemic shunt (PSS). Tumor or inflammation of hepatobiliary system was suspected as the cause of obstruction of the common bile duct. But it could not be determined without biopsy. The severely dilated cystic duct was considered to cause portal hypertension and secondary multiple PSS. The patient expired without histopathologic examination.
Animals ; Biliary Tract ; Biopsy ; Cats ; Child, Preschool ; Cholestasis, Extrahepatic ; Common Bile Duct ; Cystic Duct ; Depression ; Diagnosis ; Female ; Humans ; Hypertension, Portal ; Inflammation ; Jaundice ; Mouth Mucosa ; Physical Examination ; Portasystemic Shunt, Surgical ; Sclera ; Ultrasonography ; Vomiting ; Weight Loss

Ascites is the most common cause of decompensation in cirrhosis, and 5% to 10% of patients with compensated cirrhosis develop ascites each year. The main factor of ascites formation is renal sodium retention due to activation of the renin-angiotensin-aldosterone system and sympathetic nervous system by the reduced effective volume secondary to splanchnic arterial vasodilation. Diagnostic paracentesis is indicated in all patients with a new onset of grade 2 or 3 ascites and in those admitted to hospital for any complication of cirrhosis. A serum-ascites albumin gradient of ≥1.1 g/dL indicates portal hypertension with an accuracy of approximately 97%. Sodium restriction, diuretics, and large volume paracentesis are the mainstay of treatment in grade 1 to 3 ascites. The refractoriness of ascites is associated with a poor prognosis with a median survival of approximately six months. Repeated large volume paracentesis plus albumin is the first line treatment, and liver transplantation is recommended in patients with refractory ascites. A careful selection of patients is also important to obtain the beneficial effects of transjugular intrahepatic portosystemic shunts in refractory ascites. This review details the recent diagnosis and treatment of cirrhotic ascites.
Ascites* ; Diagnosis ; Diuretics ; Fibrosis ; Humans ; Hypertension, Portal ; Liver Cirrhosis ; Liver Transplantation ; Paracentesis ; Portasystemic Shunt, Surgical ; Prognosis ; Renin-Angiotensin System ; Sodium ; Sympathetic Nervous System ; Vasodilation

Accessory hepatic lobe is noted as and considered a rare disease in children. It can manifest with various symptoms and complications depending on the location, volume, type and position of the disease as presented on a child. The patient presented as a 14-month-old girl who was seen with a notable hepatosplenomegaly and portal hypertension. A diagnosis was made after taking an extensive medical history, observation and radiological examinations. The formal diagnosis was a prehepatic portal hypertension associated with accessory hepatic lobe.
Child* ; Diagnosis ; Female ; Humans ; Hypertension, Portal* ; Infant ; Rare Diseases

Arteriovenous fistula (AVF) involving the inferior mesenteric artery and vein is very rare with only 33 cases described in the literature and may be of congenital or acquired (iatrogenic or traumatic) or idiopathic etiology. The pathophysiology of AVF that acts as a left-to-right shunt has accounted for clinical signs and symptoms associated with ischemic colitis, portal hypertension, and heart failure. A low incidence and nonspecific clinical signs and symptoms such as abdominal pain, thrill and mass, lower and upper gastrointestinal bleeding make it difficult to establish a diagnosis of inferior mesenteric AVF. Diagnosis of inferior mesenteric AVF is usually established by radiological or intraoperative examination. We report a case of idiopathic inferior mesenteric AVF causing ischemic colitis in a 56-year-old man that was diagnosed preoperatively by multidetector computed tomography and angiography and successfully treated by surgical resection.
Abdominal Pain ; Angiography ; Arteriovenous Fistula* ; Colitis, Ischemic ; Diagnosis ; Heart Failure ; Hemorrhage ; Humans ; Hypertension, Portal ; Incidence ; Mesenteric Artery, Inferior ; Middle Aged ; Multidetector Computed Tomography ; Veins

BACKGROUNDBalanced adjustment of the portal vein shunt volume during a transjugular intrahepatic portosystemic shunt (TIPS) is critical for maintaining liver perfusion and decreasing the incidence of liver insufficiency. A stent-graft is proved to be superior to a bare metal stent (BMS) for the construction of a TIPS. However, the clinical results of the combination application of stents and stent-grafts have not been determined. This study aimed to compare the technique of using a combination of stents and stent-grafts with using a single stent-graft to construct a TIPS.

METHODSFrom April 2011 to November 2014, a total of fifty patients were randomly assigned to a stents-combination group (Group I, n = 28) or a stent-graft group (Group II, n = 22). Primary patency rates were calculated. Clinical data, including the technical success rate, bleeding control results, incidence of encephalopathy, liver function preservation, and survival rate, were assessed.

RESULTSTechnically, the success rate was 100% for both groups. The primary patency rates at 1, 2, and 3 years for Group I were 96%, 84%, and 77%, respectively; for Group II, they were 90%, 90%, and 78%, respectively. The survival rates at 1, 2, and 3 years for Group I were 79%, 74%, and 68%, respectively; for Group II, they were 82%, 82%, and 74%, respectively. The incidence of hepatic encephalopathy was 14.3% for Group I and 13.6% for Group II. The Child-Pugh score in Group I was stable at the end of the follow-up but had significantly increased in Group II (t = -2.474, P = 0.022).

CONCLUSIONSThe construction of a TIPS with either the single stent-graft or BMS/stent-graft combination is effective for controlling variceal bleeding. The BMS/stent-graft combination technique is superior to the stent-graft technique in terms of hepatic function preservation indicated by the Child-Pugh score. However, considering the clinical results of the TIPS, the two techniques are comparable in their primary shunt patency, incidence of encephalopathy and patient survival during the long-term follow-up.

Aged ; Female ; Hepatic Encephalopathy ; diagnosis ; etiology ; Humans ; Hypertension, Portal ; complications ; mortality ; surgery ; Male ; Middle Aged ; Polytetrafluoroethylene ; Portal Vein ; surgery ; Portasystemic Shunt, Transjugular Intrahepatic ; adverse effects ; methods ; Postoperative Complications ; Prospective Studies ; Stents ; Treatment Outcome

Idiopathic noncirrhotic portal hypertension is a poorly defined clinical condition of unknown etiology. Patients present with signs and symptoms of portal hypertension without evidence of cirrhosis. The disease course appears to be indolent and benign with an overall better outcome than cirrhosis, as long as the complications of portal hypertension are properly managed. This condition has been recognized in different parts of the world in diverse ethnic groups with variable risk factors, resulting in numerous terminologies and lack of standardized diagnostic criteria. Therefore, although the diagnosis of idiopathic noncirrhotic portal hypertension requires clinical exclusion of other conditions that can cause portal hypertension and histopathologic confirmation, this entity is under-recognized clinically as well as pathologically. Recent studies have demonstrated that variable histopathologic entities with different terms likely represent a histologic spectrum of a single entity of which obliterative portal venopathy might be an underlying pathogenesis. This perception calls for standardization of the nomenclature and formulation of widely accepted diagnostic criteria, which will facilitate easier recognition of this disorder and will highlight awareness of this entity.
Diagnosis ; Ethnic Groups ; Fibrosis ; Humans ; Hypertension, Portal* ; Liver ; Risk Factors

Portal biliopathy is defined as abnormalities in the extra- and intrahepatic ducts and gallbladder of patients with portal hypertension. This condition is associated with extrahepatic venous obstruction and dilatation of the venous plexus of the common bile duct, resulting in mural irregularities and compression of the biliary tree. Most patients with portal biliopathy remain asymptomatic, but approximately 10% of them advance to symptomatic abdominal pain, jaundice, and fever. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography are currently used as diagnostic tools because they are noninvasive and can be used to assess the regularity, length, and degree of bile duct narrowing. Management of portal biliopathy is aimed at biliary decompression and reducing the portal pressure. Portal biliopathy has rarely been reported in Korea. We present a symptomatic case of portal biliopathy that was complicated by cholangitis and successfully treated with biliary endoscopic procedures.
Abdomen/diagnostic imaging ; Cholangiopancreatography, Endoscopic Retrograde ; Cholestasis/*diagnosis ; Humans ; Hypertension, Portal/*diagnosis ; Male ; Middle Aged ; Portal Vein ; *Stents ; Tomography, X-Ray Computed

Neurofibromas can occur anywhere in the body, but they usually involve the head, neck, pelvis, and extremities. Abdominal visceral involvement is rare, and intrahepatic involvement is even less common. We describe a patient who suffered from plexiform neurofibromatosis with liver involvement. A 49-year-old man, who had previously been diagnosed with neurofibromatosis, underwent esophagogastroduodenoscopy and abdominal ultrasonography for screening purposes. Esophagogastroduodenoscopy showed grade 2 esophageal varices and abdominal ultrasonography showed conglomerated nodules with echogenic appearances in the perihepatic space. Magnetic resonance imaging showed presumed plexiform neurofibroma involving the lesser sac and hepatic hilum and encasing the common hepatic artery celiac trunk and superior mesenteric artery left portal triad. We report an unusual case of portal hypertension attributed to the compressive narrowing of the portal vein by presumed as plexiform neurofibroma at the lesser sac and hepatic hilum.
Abdomen/diagnostic imaging ; Endoscopy, Digestive System ; Esophageal and Gastric Varices/pathology ; Hepatic Artery/diagnostic imaging ; Humans ; Hypertension, Portal/*diagnosis ; Liver/diagnostic imaging ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neurofibroma, Plexiform/*diagnosis/diagnostic imaging ; Tomography, X-Ray Computed ; Ultrasonography