Article
Surgery
Hikmet Aktas, Ali Ozer, Tonguc Utku Yilmaz, Selim Keceoglu, Meltem Guner Can, Remzi Emiroglu
Summary: This study investigates the outcomes of liver transplantation for patients with Budd-Chiari syndrome. The results show that liver transplantation with individualized surgical and postoperative treatment strategy provides comparable outcomes, but the risk of thrombotic complications is significantly increased in the Budd-Chiari group.
ASIAN JOURNAL OF SURGERY
(2022)
Review
Medicine, General & Internal
Juan Carlos Garcia-Pagan, Dominique-Charles Valla
Summary: Primary Budd-Chiari Syndrome (BCS) is a life-threatening disease caused by obstruction of the hepatic venous outflow tract due to thrombosis or a primary venous wall disease. Recent advancements have enhanced our knowledge of BCS and its outcomes.
NEW ENGLAND JOURNAL OF MEDICINE
(2023)
Article
Surgery
Joseph J. Alukal, Talan Zhang, Paul J. Thuluvath
Summary: The study found that BCS patients listed as status 1 have excellent survival following emergency liver transplantation. There was no significant difference in long-term survival and intention-to-treat outcomes between status 1 and non-status 1 patients.
AMERICAN JOURNAL OF TRANSPLANTATION
(2021)
Article
Medicine, General & Internal
Qiao-Bo Ye, Qin-Feng Huang, Yao-Chang Luo, Yi-Lei Wen, Zi-Kun Chen, Ai-Ling Wei
Summary: BCS is a rare liver disease with nonspecific clinical features that can often be misdiagnosed, necessitating timely and accurate diagnosis and treatment to prevent progression to liver cirrhosis.
WORLD JOURNAL OF CLINICAL CASES
(2021)
Article
Health Care Sciences & Services
Yaru Tong, Ming Zhang, Zexue Qi, Wei Wu, Jinjun Chen, Fuliang He, Hao Han, Pengxu Ding, Guangchuan Wang, Yuzheng Zhuge
Summary: This study retrospectively analyzed clinical manifestations, laboratory tests, and imaging features of 139 patients with BCS-HV and 257 patients with PA-HSOS. The study found that hepatic vein collateral circulation, enlarged caudate lobe of the liver, and early liver enhancement nodules were more common in BCS-HV patients compared to PA-HSOS patients, suggesting that these imaging features are important indicators for distinguishing between the two diseases.
JOURNAL OF PERSONALIZED MEDICINE
(2023)
Article
Pathology
Hiroshi Kobayashi, Kenji Notohara, Mitsuko Nakashima, Masuo Ujita, Toru Takano, Shunsuke Tsubata, Riuko Ohashi, Hirotomo Saitsu, Souichi Sugitani
Summary: This article reports a case of Budd-Chiari syndrome (BCS) associated with hepatic small vessel neoplasia (HSVN). The patient had multiple hepatic nodules that gradually increased in size and were confirmed to be multifocal HSVN. The article discusses the relationship between BCS and HSVN.
Review
Medicine, General & Internal
Francesco Rizzetto, Davide Rutanni, Luca Alessandro Carbonaro, Angelo Vanzulli
Summary: Budd-Chiari syndrome is a rare clinical condition characterized by liver venous outflow obstruction, resulting in liver congestion and chronic parenchymal damage. It leads to the development of liver lesions, including benign focal nodular hyperplasia-like regenerative nodules, hepatocellular carcinoma, and perfusion-related pseudo-lesions. Various imaging modalities are used to monitor BCS patients and detect new lesions, but differentiating between benign and malignant nodules can be challenging due to overlapping characteristics. This review provides a comprehensive overview of the imaging features and differential diagnosis of focal liver lesions in BCS, aiming to assist clinical decision-making.
Article
Gastroenterology & Hepatology
Paul J. Thuluvath, Joseph J. Alukal, Talan Zhang
Summary: ALF due to BCS is associated with a very high in-hospital mortality that could be predicted with reasonable accuracy through a prediction model developed using clinical variables.
HEPATOLOGY INTERNATIONAL
(2021)
Review
Medicine, General & Internal
Alberto Quaglia
Summary: This article summarizes the histopathological changes in Budd-Chiari syndrome (BCS), which overlap with sinusoidal obstruction syndrome (SOS) and right cardiac failure. It is important to differentiate these conditions from BCS based on clinical and pathological grounds. The review focuses on three main aspects of BCS in diagnostic liver histopathology: general histopathology of BCS, implications for liver biopsy interpretation, and BCS in the liver allograft. The histological features of BCS vary in each individual case and sampling variation limits the use of liver biopsy for prognostication.
Review
Medicine, General & Internal
Martin Rossle
Summary: Medical treatment is limited in patients with Budd-Chiari syndrome, and most require interventional treatment. Asian countries commonly have short-segment stenosis or occlusion of hepatic veins or the inferior vena cava, treated with angioplasty or stent implantation. Western countries often have long-segment thrombotic occlusion of hepatic veins, requiring portocaval shunting procedures. The transjugular intrahepatic portosystemic shunt is gaining popularity as an effective treatment option.
Article
Medicine, General & Internal
Feng Dai, Wei Qiao, Zheng Kang, Yan Chen, Kang Li, Wenrong Shen, Xiuming Zhang
Summary: This study retrospectively analyzed the clinical features and CT manifestations of hepatic sinusoidal obstruction syndrome (HSOS) induced by Gynura segetum in 20 patients. The results showed abnormal liver function indicators and CT manifestations of ascites, heterogeneous hypoattenuation, and hepatic vein stenosis in HSOS patients.
INTERNATIONAL JOURNAL OF GENERAL MEDICINE
(2022)
Article
Medicine, General & Internal
Marius Ibach, Dennis Eurich, Eva Dobrindt, Georg Lurje, Wenzel Schoening, Robert Oellinger, Johann Pratschke, Brigitta Globke
Summary: This study evaluated the long-term outcomes of liver transplantation for Budd-Chiari syndrome (BCS) in 46 patients, showing survival rates of 87%, 83%, 76%, and 60% at 1, 5, 10, and 20 years post-transplantation. Liver transplantation for BCS demonstrated excellent results compared to other indications, with patients with myeloproliferative disorders showing lower survival rates.
MEDICINA-LITHUANIA
(2021)
Article
Radiology, Nuclear Medicine & Medical Imaging
Lu-Lu Lv, Li-Li Zhu, Gao-Hong Chen, Peng Xu, Kai Xu
Summary: The study showed that recanalization of hepatic vein-type Budd-Chiari syndrome via accessory hepatic vein achieved similar technical and clinical success rates compared to main hepatic vein recanalization, but had higher long-term patency rates.
ABDOMINAL RADIOLOGY
(2021)
Article
Integrative & Complementary Medicine
Yogesh Narayan Bendale, Vineeta Bendale, Avinash Kadam, Poonam Birari-Gawande, Ketaki Jagtap
Summary: This article reports the successful treatment of a female patient with Budd-Chiari syndrome using exclusively herbo-mineral Ayurvedic medicines. The treatment resulted in the restoration of health, normalization of liver function, and regression of thromboses. This case study provides primary evidence of the potential of Ayurveda in improving therapeutic outcomes in patients with BCS.
ALTERNATIVE THERAPIES IN HEALTH AND MEDICINE
(2023)
Article
Gastroenterology & Hepatology
Chaoyang Wang, Tongqiang Li, Kequan Chen, Huanzhang Niu, Yaowei Bai, Jiacheng Liu, Yingliang Wang, Shuguang Ju, Wei Yao, Guorui Zhao, Bin Xiong, Guofeng Zhou
Summary: EV treatment can improve liver function, alleviate liver cirrhosis and portal hypertension in Chinese patients with BCS. It can restore liver architecture, inhibit hepatic angiogenesis and inflammation, and shift the state of hepatocytes from apoptosis to proliferation.
HEPATOLOGY RESEARCH
(2023)
Article
Pathology
Luis Veloza, Chun-Yi Tsai, Bettina Bisig, Olivier Pantet, Lorenzo Alberio, Christine Sempoux, Matthias Cavassini, Laurence De Leval
Summary: TRAIL, a novel protein for tumor therapy, can induce apoptosis of tumor cells and effectively inhibit the growth and spread of various tumors, as confirmed by clinical trials.
Article
Hematology
Laurence de Leval, Ash A. Alizadeh, P. Leif Bergsagel, Elias Campo, Andrew Davies, Ahmet Dogan, Jude Fitzgibbon, Steven M. Horwitz, Ari M. Melnick, William G. Morice, Ryan D. Morin, Bertrand Nadel, Stefano A. Pileri, Richard Rosenquist, Davide Rossi, Itziar Salaverria, Christian Steidl, Steven P. Treon, Andrew D. Zelenetz, Ranjana H. Advani, Carl E. Allen, Stephen M. Ansell, Wing C. Chan, James R. Cook, Lucy B. Cook, Francesco D'Amore, Stefan Dirnhofer, Martin Dreyling, Kieron Dunleavy, Andrew L. Feldman, Falko Fend, Philippe Gaulard, Paolo Ghia, John G. Gribben, Olivier Hermine, Daniel J. Hodson, Eric D. Hsi, Giorgio Inghirami, Elaine S. Jaffe, Kennosuke Karube, Keisuke Kataoka, Wolfram Klapper, Won Seog Kim, Rebecca L. King, Young H. Ko, Ann S. LaCasce, Georg Lenz, Jose Martin-Subero, Miguel A. Piris, Stefania Pittaluga, Laura Pasqualucci, Leticia Quintanilla-Martinez, Scott J. Rodig, Andreas Rosenwald, Gilles A. Salles, Jesus San-Miguel, Kerry J. Savage, Laurie H. Sehn, Gianpietro Semenzato, Louis M. Staudt, Steven H. Swerdlow, Constantine S. Tam, Judith Trotman, Julie M. Vose, Oliver Weigert, Wyndham H. Wilson, Jane N. Winter, Catherine J. Wu, Pier L. Zinzani, Emanuele Zucca, Adam Bagg, David W. Scott
Summary: With the introduction of large-scale molecular profiling methods and high-throughput sequencing technologies, the genomic features of most lymphoid neoplasms have been characterized at an unprecedented scale. However, the current diagnosis of lymphoid tumors is largely based on morphological assessment and immunophenotyping, with only few entities being defined by genomic criteria. This paper discusses how established and newly developed molecular testing technologies complement clinical diagnoses and contribute to refining diagnosis, risk stratification, and therapy prediction for lymphoid neoplasms. Future methods such as whole-genome sequencing, circulating tumor DNA analyses, single-cell analyses, and epigenetic profiling are likely to become important tools for implementing precision medicine approaches in clinical decision making for lymphoid malignancies.
Review
Pathology
Andrew L. Feldman, Camille Laurent, Marina Narbaitz, Shigeo Nakamura, Wing C. Chan, Laurence de Leval, Philippe Gaulard
Summary: This article discusses the classification and diagnostic evaluation of nodal T- and NK-cell lymphomas based on the 2022 International Consensus Classification of Mature Lymphoid Neoplasms (2022 ICC). It highlights the grouping of T-follicular helper cell lymphoma into a single entity with three subtypes, as well as the genetic subtype of ALK-negative anaplastic large cell lymphoma (ALCL) associated with DUSP22 rearrangement. The introduction of a new provisional entity, primary nodal EBV-positive T-/NK-cell lymphoma, is also mentioned, along with evolving molecular data indicating distinct subgroups within PTCL, NOS.
Editorial Material
Surgery
Maud Neuberg, Olivier Detry
ACTA CHIRURGICA BELGICA
(2023)
Meeting Abstract
Hematology
Jehan Dupuis, Kunihiro Tsukasaki, Emmanuel Bachy, Franck Morschhauser, Guillaume Cartron, Noriko Fukuhara, Nicolas Daguindau, Rene-Olivier Casasnovas, Sylvia Snauwaert, Remy Gressin, Christopher P. Fox, Francesco Annibale d'Amore, Philipp B. Staber, Argyrios Gkasiamis, Mitsufumi Nishio, Luc-Matthieu Fornecker, Marie-Helene Delfau, Nouhoum Sako, Sebastien Mule, Laurence De Leval, Philippe Gaulard, Francois Lemonnier
Article
Pathology
Come Sesboue, Jean Galtier, Marie Jeanneau, Annick Chauvel, Elodie Laharanne, Samuel Amintas, Jean-Philippe Merlio, Krimo Bouabdallah, Francois-Xavier Gros, Laurence de Leval, Audrey Gros, Marie Parrens
Summary: Distinguishing FL from NMZL can be difficult in cases with unusual morphologic and phenotypic features and without characteristic cytogenetic rearrangements. Ancillary techniques, such as FISH, RT-MLPA, and NGS, can provide diagnostic contributions for unclassified tumors. In this study, FISH detected 1p36 deletion in some FLs and unclassified tumors, while RT-MLPA signatures were associated with FL and NMZL subtypes. Combining RT-MLPA and NGS findings successfully discriminated most unclassified tumors towards FL or NMZL diagnosis.
Article
Pathology
Rita Sarkis, Olivier Burri, Claire Royer-Chardon, Frederica Schyrr, Sophie Blum, Mariangela Costanza, Stephane Cherix, Nathalie Piazzon, Carmen Barcena, Bettina Bisig, Valentina Nardi, Rossella Sarro, Giovanna Ambrosini, Martin Weigert, Olivier Spertini, Sabine Blum, Bart Deplancke, Arne Seitz, Laurence de Leval, Olaia Naveiras
Summary: Bone marrow cellularity assessment is crucial in evaluating bone marrow biopsies for hematologic and nonhematologic disorders. MarrowQuant 2.0 is an efficient digital hematopathology workflow that accurately quantifies the cellularity of human bone marrow biopsies by segmenting different compartments. This tool can be used for clinical research to explore biomarkers related to bone marrow stromal components.
Review
Pathology
Laurence de Leval, Andrew L. Feldman, Stefano Pileri, Shigeo Nakamura, Philippe Gaulard
Summary: Non-cutaneous extranodal NK/T cell lymphoproliferations are a rare group of tumors that primarily occur in the gastrointestinal tract, nasal area, spleen, and liver. Accurate diagnosis is crucial due to the diverse clinical presentations and similarities with other diseases.
Article
Surgery
Clara Bihain, Jean Delwaide, Paul Meunier, Laurent Gerard, Alexandre Jadoul, Olivier Detry
Summary: The case report describes a large hepatocellular carcinoma (HCC) found in a non-cirrhotic liver. The patient underwent selective internal radiation therapy and surgery, showing no signs of HCC recurrence after 30 months. The discussion highlights the better prognosis of HCC in non-cirrhotic liver and the challenges of late diagnosis and inadequate screening.
ACTA CHIRURGICA BELGICA
(2023)
Review
Pathology
Leticia Quintanilla-Martinez, Camille Laurent, Lorinda Soma, Siok-Bian Ng, Fina Climent, Sarah L. L. Ondrejka, Alberto Zamo, Andrew Wotherspoon, Laurence de Leval, Stefan Dirnhofer, Lorenzo Leoncini
Summary: The 2022 European Association for Haematopathology/Society for Hematopathology workshop in Florence, Italy discussed emerging entities and molecular subgroups in large B-cell lymphomas (LBCLs). The session focused on newly recognized diseases and their diagnostic challenges. Topics included high-grade/large B-cell lymphoma with 11q aberration (HG/LBCL-11q), LBCL with IRF4 rearrangement (LBCL-IRF4), primary bone diffuse large B-cell lymphoma (PB-DLBCL), and B-acute lymphoblastic leukemia (B-ALL) with IGH::MYC translocation.
Review
Pathology
Alberto Zamo, Michiel van den Brand, Fina Climent, Laurence de Leval, Stefan Dirnhofer, Lorenzo Leoncini, Siok-Bian Ng, Sarah L. Ondrejka, Leticia Quintanilla-Martinez, Lorinda Soma, Andrew Wotherspoon
Summary: Session 3 of the lymphoma workshop at the joint meeting of the European Association for Haematopathology and the Society for Hematopathology discussed splenic and nodal marginal zone lymphomas, transformation in marginal zone lymphomas, and pediatric nodal marginal zone lymphomas. The case review emphasized the challenges in diagnosing marginal zone lymphomas, including distinguishing between different types and the need for molecular studies to establish a final diagnosis. The workshop also addressed the overlapping spectrum between pediatric nodal marginal zone lymphoma and pediatric-type follicular lymphoma.
Correction
Pathology
Alberto Zamo, Michiel van den Brand, Fina Climent, Laurence de Leval, Stefan Dirnhofer, Lorenzo Leoncini, Siok-Bian Ng, Sarah L. Ondrejka, Leticia Quintanilla-Martinez, Lorinda Soma, Andrew Wotherspoon
Review
Pathology
Fina Climent, Alina Nicolae, Laurence de Leval, Stefan Dirnhofer, Lorenzo Leoncini, Sarah L. Ondrejka, Lorinda Soma, Andrew Wotherspoon, Alberto Zamo, Leticia Quintanilla-Martinez, Siok-Bian Ng
Summary: The 2022 European Association for Haematopathology/Society for Hematopathology lymphoma workshop discussed cytotoxic peripheral T-cell lymphomas and EBV-positive T/NK-cell lymphoproliferative diseases. Primary nodal-EBV-TNKL is a newly recognized entity associated with underlying immune deficiency/immune dysregulation. cPTCL-NOS demonstrates the TBX21 phenotype and shows an unexpected association with TFH lymphomas/LPDs.
Article
Hematology
Kamel Laribi, Alix Baugier de Materre, Yamina Touileb, Charles Boursot, Jeremy Sandrini, Doriane Cavalieri, Cedric Pastoret, Laurence de Leval, Olivier Tournilhac
Letter
Hematology
Roch Houot, Viola Poeschel, Bettina Altmann, Stephanie Angel, Lorenz Thurner, Thomas Illmer, Marc Andre, Martin Dreyling, Herve Maisonneuve, Herve Tilly, Stephanie Mayer, Olivier Casasnovas, Steven Le Gouill, Fritz Offner, Guillaume Cartron, Andrea Kerkhoff, Thomas Weber, Joerg Hoffmann, Marita Ziepert, Wolfram Klapper, Emmanuel Itti, Dirk Hellwig, Giorgi Natchkebia, Laurence de Leval, Andreas Rosenwald, Corinne Haioun, Laurent Dercle, Philippe Gaulard, Gerhard Held
