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Systemic mastocytosis: A rare cause of non-cirrhotic portal hypertension

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World Journal of Gastroenterology 2016年第28期22卷 6559-6564页

作者： Cláudio Martins Cristina Teixeira Suzane Ribeiro Daniel Trabulo Cláudia Cardoso Joao Mangualde Ricardo Freire élia Gamito Ana Luísa Alves Isabelle Cremers Cecília Alves Anabela Neves Ana Paula Oliveira Department of Gastroenterology Setubal Hospital Center - Sao Bernardo Hospital 2910 Setubal Portugal Department of Pathology Setubal Hospital Center - Sao Bernardo Hospital 2910 Setubal Portugal Department of Hematology Setubal Hospital Center - Sao Bernardo Hospital 2910 Setubal Portugal

Mastocytosis is a clonal neoplastic disorder of the mast cells(MC) that can be limited to the skin(cutaneous mastocytosis) or involve one or more extracutaneous organs(systemic mastocytosis). The clinical manifestations of mastocytosis are heterogeneous ranging from indolent disease with a long-term survival to a highly aggressive neoplasm with survival of about 6 mo. Although liver involvement in aggressive systemic mastocytosis(ASM) is relatively common, the development of portal hypertension with or without cirrhosis is rare. We report a case of ASM without skin involvement in a 72-year-old caucasian male who presented with non-cirrhotic portal hypertension based on clinical, analytical, imagiological and endoscopic findings. Given the hematological picture, the correct diagnosis was established based on ancillary tests for MC using bone marrow aspirates and biopsy. Extensive involvement of the liver and gastrointestinal tract was histologically documented. The disease progressed rapidly and severe pancytopenia and recurrent upper gastrointestinal bleeding became the dominant problem. This case illustrates the challenge in establishing a diagnosis of ASM especially when the clinical picture is atypical and without skin involvement. Gastroenterologists should consider infiltrative disease, particularly systemic mastocytosis, as a differential diagnosis in a clinical case of portal hypertension of unknown etiology.

关键词： Systemic mastocytosis Mast cells non-cirrhotic portal hypertension Upper gastrointestinal bleeding Cladribine

Isolated gastric variceal bleeding related to non-cirrhotic portal hypertension following oxaliplatin-based chemotherapy:A case report

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World Journal of Gastroenterology 2022年第27期28卷 3524-3531页

作者： Xu Zhang Yan-Ying Gao De-Zhao Song Bao-Xin Qian Department of Gastroenterology and Hepatology The Third Central Hospital of TianjinTianjin Key Laboratory of Extra-corporeal Life Support for Critical DiseasesArtificial Cell Engineering Technology Research CenterTianjin Institute of Hepatobiliary DiseaseTianjin 300000China Department of Interventional Radiology The Third Central Hospital of TianjinTianjin Key Laboratory of Extracorporeal Life Support for Critical DiseasesArtificial Cell Engineering Technology Research CenterTianjin Institute of Hepatobiliary DiseaseTianjin 300000China Department of Gastroenterology and Hepatology The Third Central Hospital of TianjinTianjin Key Laboratory of Extracorporeal Life Support for Critical DiseasesArtificial Cell Engineering Technology Research CenterTianjin Institute of Hepatobiliary DiseaseTianjin 300000China

BACKGROUND Sinusoidal obstruction syndrome has been reported after oxaliplatin-based chemotherapy,but liver fibrosis and non-cirrhotic portal hypertension(NCPH)are rarely *** SUMMARY Here,we describe the case of a 64-year-old woman who developed isolated gastric variceal bleeding 16 mo after completing eight cycles of oxaliplatin combined with capecitabine chemotherapy after colon cancer ***,splenomegaly and thrombocytopenia were not accompanied by variceal bleeding,which has been reported to have predictive value for gastric variceal ***,a liver biopsy showed fibrosis in the portal area,suggesting *** patient underwent endoscopic treatment and experienced no further *** It is necessary to guard against long-term complications after oxaliplatin-based *** splenic size and platelet level may not always accurately predict the occurrence of portal hypertension.

关键词： non-cirrhotic portal hypertension Sinusoidal obstruction syndrome Isolated gastric varices Oxaliplatin chemotherapy Colon cancer Case report

Pathological abnormalities in splenic vasculature in non-cirrhotic portal hypertension:Its relevance in the management of portal hypertension

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World Journal of Gastrointestinal Surgery 2020年第1期12卷 1-8页

作者： Shahana Gupta Biju Pottakkat Surendra Kumar Verma Raja Kalayarasan Sandip Chandrasekar A Ajith Ananthakrishna Pillai Department of Surgical Gastroenterology JIPMERPondicherry 605006India Department of Pathology JIPMERPondicherry 605006India Department of Cardiology JIPMERPondicherry 605006India

BACKGROUND portal hypertension(PH)is associated with changes in vascular structure and function of the portosplenomesenteric system(PSMS).This is referred to as portal hypertensive *** abnormalities of PSMS has been described in the literature for cirrhotic *** portal pressure and hyperdynamic circulation are thought to be the underlying cause of this *** view of this,it is expected that pathological changes in splenic and portal vein similar to those reported in cirrhotic patients with PH may also be present in patients with non-cirrhotic PH(NCPH).AIM To investigate pathological abnormalities of splenic vein in patients with NCPH,and suggest its possible implications in the management of *** A prospective observational study was performed on 116 patients with NCPH[Extrahepatic portal vein obstruction(EHPVO):53 and non-cirrhotic portal fibrosis(NCPF):63]who underwent proximal splenorenal shunt(PSRS),interposition shunt or splenectomy with devascularization in JIPMER,Pondicherry,India,a tertiary level referral center,between *** patients were evaluated by Doppler study of PSMS,computed tomography portovenogram and upper gastrointestinal *** acoustic resonance forced impulse(ARFI)scan and abdomen ultrasound were done for all cases to exclude *** and histopathological assessment of the harvested splenic vein was performed in *** study group was divided into delayed and early presentation based on the median duration of symptoms(i.e.108 mo).RESULTS The study group comprising of 116 patients[77(66%)females and 39(34%)males]with NCPH had a median age of 22 *** duration of symptoms was 108 *** most common presentation in both EHPVO and NCPF patients was upper gastrointestinal bleeding(hematemesis and melena).The ARFI scan revealed a median score of 1.2(1.0-1.8)m/s for EHPVO and 1.5(0.9-2.8)m/s for *** was performed in 84 patients(two of whom underwent interpos

关键词： portal hypertensive vasculopathy non-cirrhotic portal hypertension Splenic vasculature Hyperdynamic circulation Shunt surgery

A rabbit model of non- cirrhotic portal hypertension by repeated injections of E. coli through indwelling cannulation of the gastrosplenic vein

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Hepatobiliary & Pancreatic Diseases International 2004年第3期3卷 417-422页

作者： Swati Omanwar Moattar R. Rizvi Rachna Kathayat Brij K. Sharma Giryesh K. Pandey Mohammad A. Alam Veena Malhotra Shiv K. Sarin Departments of Gastroenterology G. B. Pant Hospital New Delhi India Departments of Pathology G. B. Pant Hospital New Delhi India

BACKGROUND: non-cirrhotic portal hypertension is acommon cause of portal hypertension in developing coun-tries. To understand its etiopathogenesis we developed ananimal model by repeated portal endotoxemia inducedthrough the gastrosplenic ***: Twenty-nine rabbits (1.5-2.0 kg) were divid-ed into control (group n = 13) and experimental ( groupn = 16) groups. Heat killed E. coli were injected throughan indwelling cannula into the gastrosplenic vein in pre-sensitized animals. The animals were sacriflced at 1, 3 and6 ***: The mean portal pressure in group animalswas significantly (P portal lympho-cytic infiltrates and kupffer cell hyperplasia. No significantanomalies were detected by liver function ***: The rabbit model showed significantsplenomegaly with a persistent increase in portal pressureand mild fibrosis without hepatic parenchymal injury, quiteakin to non-cirrhotic portal fibrosis as seen in humans. Re-current intra-abdominal infection may play an importantrole in the pathogenesis of non-cirrhotic portal fibrosis.

关键词： non-cirrhotic portal fibrosis non-cirrhotic portal hypertension portal hypertension variceal bleeding portal pressure animal models idiopathic portal hypertension

Application of ultrasonography-elastography score to suspect porto-sinusoidal vascular disease in patients with portal vein thrombosis

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Hepatobiliary & Pancreatic Diseases International 2024年第1期23卷 20-24页

作者： Stefania Gioia Adriano De Santis Giulia d’Amati Silvia Nardelli Alessandra Spagnoli Arianna Di Rocco Lorenzo Ridola Oliviero Riggio Department of Translational and Precision Medicine Sapienza University of RomeRomeItaly Department of Radiological Oncologicaland Pathological SciencesSapienza University of RomeRomeItaly Department of Public Health and Infectious Diseases Sapienza University of RomeRomeItaly

Background:Porto-sinusoidal vascular disease(PSVD)and portal vein thrombosis(PVT)are causes of portal hypertension characterized respectively by an intrahepatic and a pre-hepatic obstacle to the flow in the portal *** PVT may be a consequence of PSVD,in PVT patients at presentation,a pre-existing PSVD should be *** these patients the identification of an underlying PSVD would have relevant implication regarding follow-up and therapeutic management,but it could be *** this setting ultrasonography may be valuable in differential *** aim of the study was to use ultrasonography to identify parameters to discriminate between PSVD and“pure”PVT and then to suspect PVT secondary to a pre-existing ***:Fifty-three patients with histologically proven PSVD and forty-eight patients affected by chronic PVT were enrolled and submitted to abdominal ultrasonography with elastography by acoustic radiation force impulse(ARFI).Results:ARFI was higher and superior mesenteric vein(SMV)diameter was wider in PSVD patients than in PVT ***,a prognostic score was obtained as linear combinations of the two parameters with a good discrimination capacity between PSVD and PVT(the area under the curve=0.780;95%confidence interval:0.690-0.869).Conclusions:A score based on ARFI and SMV diameter may be useful to suspect an underlying PSVD in patients with PVT and to identify a subgroup of patients to be submitted to liver biopsy.

关键词： non-cirrhotic portal hypertension Porto-sinusoidal vascular disease Chronic portal vein thrombosis Liver stiffness portal hypertension Acoustic radiation force impulse

New guidelines for screening,evaluation and management of hepatobiliary disease in cystic fibrosis

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Hepatobiliary Surgery and Nutrition 2024年第5期13卷 875-878页

作者： P.Aiden McCormick Emer Fitzpatrick Liver Unit St Vincent’s University HospitalDublinIreland Department of Medicine UCD School of MedicineUniversity College DublinDublinIreland Gastroenterology and Liver Department Children’s Health IrelandCrumlinDublinIreland

The recently published guidelines on screening,monitoring and treatment update previous guidelines published 25 years ago(1,2).This update is timely as much has happened in the *** fibrosis(CF)modulator therapy has dramatically improved prognosis for patients with CF,although it is not yet clear whether liver disease is *** understanding of the pathophysiology of CF liver disease has also changed with the recognition of the importance of non-cirrhotic portal hypertension(3,4).non-invasive tests for fibrosis and elastography have changed the practice of hepatology and reduced the need for liver *** guideline committee was made up of experts from North America and Europe and included adult and paediatric hepatologists and pulmonologists together with allied health practitioners and representatives of the CF community.A systematic literature search was performed and a vote of 80%was required to adopt a *** guidelines contain 7 recommendations for screening,13 for disease monitoring and 14 for treatment.

关键词： Cirrhosis oesophageal varices non-cirrhotic portal hypertension porto-sinusoidal vascular disease hepatobiliary

portal ductopathy:Clinical importance and nomenclature

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World Journal of Gastroenterology 2011年第11期17卷 1410-1415页

作者： Yusuf Bayraktar Department of Internal Medicine Gastroenterology ClinicHacettepe University HospitalsAnkara 06100Turkey

non-cirrhotic portal hypertension(PHT)accounts for about 20%of all PHT cases,portal vein thrombosis(PVT) resulting in cavernous transformation being the most common *** known complications of PHT may be encountered in patients with chronic ***,the effect of this entity on the biliary tree and pancreatic duct has not yet been fully ***,a dispute remains regarding the nomenclature of common bile duct abnormalities which occur as a result of chronic *** many clinical reports have focused on biliary abnormalities,only a few have evaluated both the biliary and pancreatic ductal *** this review the relevant literature evaluating the effect of PVT on both ductal systems is discussed,and findings are considered with reference to results of a prominent center in Turkey,from which the term"portal ductopathy"has been put forth to replace"portal biliopathy".

关键词： portal hypertension portal vein thrombosis portal vein cavernous transformation Congenital hepatic fibrosis non-cirrhotic portal hypertension portal ductopathy portal double ductopathy portal biliopathy

Gastroesophageal varices in a patient presenting with essential thrombocythemia:A case report

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World Journal of Clinical Cases 2021年第8期9卷 1871-1876页

作者： Jian-Bo Wang Yang Gao Jun-Wei Liu Mu-Gen Dai Shang-Wen Yang Bin Ye Department of Gastroenterology Lishui Hospital of Zhejiang UniversityThe Fifth Affiliated Hospital of Wenzhou Medical UniversityLishui Central HospitalLishui 323000Zhejiang ProvinceChina Department of Radiology Lishui Hospital of Zhejiang UniversityThe Fifth Affiliated Hospital of Wenzhou Medical UniversityLishui Central HospitalLishui 323000Zhejiang ProvinceChina

BACKGROUND Gastroesophageal varices are a rare complication of essential thrombocythemia(ET).ET is a chronic myeloproliferative neoplasm(MPN)characterized by an increased number of blood *** SUMMARY A 46-year-old woman,who denied a history of liver disease,was admitted to our hospital on presentation of *** examination revealed a hemoglobin level of 83 g/L,and a platelet count of 397×109/*** appearance of gastric and esophageal varices with red colored signs as displayed by an urgent endoscopy was followed by endoscopic variceal ligation and endoscopic tissue *** computed tomography revealed cirrhosis,marked splenomegaly,portal vein thrombosis and portal *** addition,bone marrow biopsy and evidence of mutated Janus kinase 2,substantiated the onset of *** patient was asymptomatic with regular routine blood testing during the 6-mo follow-up ***,in this case,gastroesophageal varices were induced by *** MPN should be given considerable attention when performing differential diagnoses in patients with gastroesophageal *** integrated approach such as laboratory tests,radiological examination,and pathological biopsy,should be included to allow optimal decisions and management.

关键词： Gastroesophageal varices Essential thrombocythemia non-cirrhotic portal hypertension Endoscopic variceal ligation Endoscopic tissue adhesive Case report

Prevalence of significant liver disease in human immunodeficiency virus-infected patients exposed to Didanosine: A cross sectional study

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World Journal of Hepatology 2016年第36期8卷 1623-1628页

作者： Sarah Logan Alison Rodger Laura Maynard-Smith James O'Beirne Thomas Fernandez Filippo Ferro Colette Smith Sanjay Bhagani the Ian Charleson Day Centre Ground Floor Royal Free London NHS Foundation Trust Research Department of Infection and Population Health University College London Department of Hepatology Nambour General Hospital Sunshine Coast Hospital and Health Service

AIM To identify significant liver disease [including nodular regenerative hyperplasia(NRH)] in asymptomatic Didanosine(DDI) exposed human immunodeficiency virus(HIV) positive *** Patients without known liver disease and with > 6 mo previous DDI use had liver stiffness assessed by transient elastography(TE). Those with alanine transaminase(ALT) above upper limit normal and/or TE > 7.65 k Pa underwent ultrasound scan(U/S). Patients with:(1) abnormal U/S; or(2) elevated ALT plus TE > 7.65 k Pa;or(3) TE > 9.4 k Pa were offered trans-jugular liver biopsy(TJLB) with hepatic venous pressure gradient(HVPG) *** Ninety-nine patients were recruited, median age 50 years(range 31-70), 81% male and 70% men who have sex with men. Ninety-five percent with VL 9.4 k Pa. Seventeen(17%) met criteria for TJLB, of whom 12 accepted. All had HVPG < 6 mm Hg. Commonest histological findings were steatosis(n = 6), normal architecture(n = 4) and NRH(n = 2), giving a prevalence of previously undiagnosed NRH of 2%(95%CI: 0.55%, 7.0%).CONCLUSION A screening strategy based on TE, liver enzymes and U/S scan found a low prevalence of previously undiagnosed NRH in DDI exposed, asymptomatic HIV positive patients. Patients were more likely to have steatosis highlighting the increased risk of multifactorial liver disease in this population.

关键词： Nodular regenerative hyperplasia Human immunodeficiency virus Steatosis non-cirrhotic portal hypertension Didanosine