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Hepatic veno occlusive disease

B K Straub, D A Ridder, A Schad, C Loquai, J M Schattenberg
BACKGROUND: Drug-induced liver injury is increasing, especially in elderly patients with polymedication and multimorbidity. OBJECTIVES: Clinicopathologic correlation of immune-mediated liver injury, specifically liver injury following therapy with immune checkpoint inhibitors against PD-1, PDL-1, and CTLA4. METHODS: Histologic assessment of liver biopsies of nine patients after therapy with immune checkpoint inhibitors and correlation with clinical parameters...
October 11, 2018: Der Pathologe
Paul G Richardson, Stephan A Grupp, Antonio Pagliuca, Amrita Krishnan, Vincent T Ho, Selim Corbacioglu
Hepatic veno-occlusive disease, also called sinusoidal obstruction syndrome (VOD/SOS), is a potentially life-threatening and unpredictable complication of hematopoietic stem cell transplantation (HSCT). Characterized by a prothrombotic-hypofibrinolytic state, VOD/SOS typically presents with hyperbilirubinemia, ascites, weight gain and painful hepatomegaly; VOD/SOS with multiorgan failure may be associated with >80% mortality. Treatment has been mainly supportive. However, defibrotide is now approved in the USA for treatment of hepatic VOD/SOS with renal or pulmonary dysfunction following HSCT and in the European Union for treatment of severe hepatic VOD/SOS post-HSCT...
November 2017: International Journal of Hematologic Oncology
Sophia Delicou, Konstantinos Maragkos, Maria Tambaki, Dimitrios Kountouras, John Koskinas
Sickle cell disease patients often need regular blood transfusions to improve both the quality of life and survival from the veno-occlusive complications of the disease. Deferasirox, a convenient long acting oral agent, has recently been introduced in clinical practice with promising efficacy. This study aims to evaluate the association of liver stiffness and possible fibrosis with iron deposition and confirm the use of elastography as a validated test of responding to chelation with low cost and easy access...
2018: Mediterranean Journal of Hematology and Infectious Diseases
Xinke Zhang, Xue Song, Lorena Lopez-Gonzalez, Krutika Jariwala-Parikh, Vadim Romanov, Ze Cong
PURPOSE: The goal of this study was to evaluate the incidence, inpatient mortality, and economic burden of hepatic veno-occlusive disease (VOD) in adults with B-cell acute lymphoblastic leukemia (ALL) in the United States. METHODS: Using MarketScan Commercial Claims and Encounters Database and Medicare Supplemental and Coordination of Benefits Database, data for patients with B-cell ALL from April 1, 2009, to October 31, 2016, were extracted by using diagnosis codes...
September 6, 2018: Clinical Therapeutics
Tal Schechter, Evelio Perez-Albuerne, Tiffany F Lin, Meredith S Irwin, Mohammed Essa, Ami V Desai, Haydar Frangoul, Gregory Yanik, L Lee Dupuis, David Jacobsohn, Morris Kletzel, Mark Ranalli, Sandeep Soni, Alix E Seif, Stephan Grupp, Christopher C Dvorak
Survival for high-risk neuroblastoma patients is still suboptimal. Although stem cell transplantation (SCT) is used, there is no consensus as to which conditioning regimen has the greatest efficacy and fewest toxicities. We assessed the incidence of and risk for hepatic veno-occlusive disease (VOD) for neuroblastoma patients who underwent autologous SCT with busulfan and melphalan (BuMel) at eight centers following Children's Oncology Group (COG)-based induction chemotherapy. Data regarding the patients, SCT characteristics, busulfan steady-state concentrations, incidence of VOD, and survival were evaluated...
September 4, 2018: Bone Marrow Transplantation
Lindsey E Roeker, Haesook T Kim, Brett Glotzbecker, Prashant Nageshwar, Sarah Nikiforow, John Koreth, Philippe Armand, Corey Cutler, Edwin P Alyea, Joseph H Antin, Paul G Richardson, Robert J Soiffer, Vincent T Ho
Hepatic veno-occlusive disease (VOD), or sinusoidal obstruction syndrome (SOS), is a serious complication of hematopoietic stem cell transplantation (HSCT) with mortality in its severe form exceeding 80%. Although the incidence of VOD/SOS has fallen with contemporary transplantation practices, the increasing use of inotuzumab, the return of gemtuzumab, and the popularity of pharmacokinetic-guided high-dose busulfan may impact incidence. Early intervention with defibrotide improves survival, but prompt diagnosis can be difficult...
August 3, 2018: Biology of Blood and Marrow Transplantation
W Liu, Y Li, Z X Qiu, Y Yin, Y H Sun, W L Xu, Q Wang, Z Y Liang, Y J Dong, L H Wang, Q N Cen, M J Wang, W S Wang, J P Ou, H Y Ren
Objective: To investigate the therapeutic effects of allogeneic hematopoietic stem cell transplantation (allo-HSCT) with FLAG sequential busulfan/cyclophosphamide(Bu/Cy) conditioning regimen for refractory/relapsed acute myeloid leukemia. Methods: From February 2012 to June 2017, 21 patients with refractory/relapsed acute myeloid leukemia underwent allo-HSCT with FLAG sequential Bu/Cy conditioning regimen. Transplantation-related complications and clinical outcome were retrospectively analyzed. Results: After conditioning, no hepatic veno-occlusive disease (VOD) and grade Ⅲ hemorrhagic cystitis occurred...
August 1, 2018: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
C J Qin, L J Liu, Z M Zhang, L Luo, Y R Lai, Q C Li
Objective: To analyze the outcome and the prognostic factors of hepatic veno-occlusive disease (HVOD) after hematopoietic stem cell transplantation (HSCT). Methods: A total of 797 patients receiving HSCT were analyzed retrospectively. The prophylaxis regimen of HVOD in the First Affiliated Hospital of Guangxi Medical University consisted of low molecular weight heparin and lipoprostaglandin E1 (PGE1). Results: Fifty-nine patients (7.4%) developed HVOD at 3-49 days after HSCT (median 12 days). Age younger than 15 years at transplant( HR = 6...
July 1, 2018: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
Emmanuelle Tavernier, Emilie Chalayer, Jérôme Cornillon, Anne Pouvaret, Jean-Alain Martignoles, François Casteillo, Jérémy Terreaux, Elisabeth Daguenet, Denis Guyotat
BACKGROUND: Hepatic veno-occlusive disease, also called sinusoidal obstruction syndrome (SOS/VOD), is a potentially fatal complication of allogeneic or autologous hematopoietic stem cell transplantation. A plethora of transplant and patient-related risk factors predispose to SOS/VOD and should be taken into account for prognosis assessment as well as for adequate therapeutic intervention. CASE PRESENTATION: We describe the case of a mantle cell lymphoma patient who developed a fulminant hepatitis following oxaliplatin-containing intensive chemotherapy and autologous transplantation...
July 3, 2018: BMC Research Notes
Paul G Richardson, Enric Carreras, Massimo Iacobelli, Bijan Nejadnik
Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is a potentially life-threatening complication of conditioning during hematopoietic stem cell transplantation (HSCT) or chemotherapy without HSCT, with a historically reported mean incidence of 13.7% post-HSCT. Typical symptoms of VOD/SOS may include hyperbilirubinemia, painful hepatomegaly, weight gain, and ascites. Defibrotide, a polydisperse mixture of predominantly single-stranded polydeoxyribonucleotides, is currently the only therapy approved to treat hepatic VOD/SOS with pulmonary/renal dysfunction (ie, multiorgan dysfunction/multiorgan failure [MOD/MOF]) following HSCT in the United States and to treat severe hepatic VOD/SOS post-HSCT in the European Union...
June 26, 2018: Blood Advances
Christopher Strouse, Ying Zhang, Mei-Jie Zhang, Alyssa DiGilio, Marcelo Pasquini, Mary M Horowitz, Stephanie Lee, Vincent Ho, Muthalagu Ramanathan, Wichai Chinratanalab, Alison Loren, Linda J Burns, Andrew Artz, Kathleen F Villa, Wael Saber
A risk score identifying patients at high risk for veno-occlusive disease (VOD) may aid efforts to study preventive strategies for this uncommon complication of hematopoietic cell transplantation (HCT). Patients receiving a first allogeneic HCT between 2008 and 2013 as reported to the Center for International Blood and Marrow Transplant Research (N = 13,097) were randomly divided into training and validation sets. Independent prognostic factors for development of VOD by day +100 after HCT were identified with a multivariate logistic regression model...
June 19, 2018: Biology of Blood and Marrow Transplantation
Farhad Khimani, George B McDonald, Howard M Shulman, Brian Betts, Frederick Locke, Hugo Fernandez, Claudio Anasetti, Joseph Pidala
Sirolimus-based graft vs. host disease (GVHD) prophylaxis is associated with higher incidence of veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) after allogeneic hematopoietic cell transplantation (HCT). However, whether the clinical manifestations and prognosis of VOD/SOS differs when diagnosed in the setting of sirolimus-based GVHD prophylaxis is not well studied. To address this question, we examined presenting features and treatment outcome of VOD/SOS cases identified in a large retrospective cohort of consecutive HCT procedures (n = 818 total, sirolimus (SIR)/tacrolimus (TAC) n = 308, and methotrexate (MTX) or mycophenolate mofetil (MMF)/TAC n = 510)...
June 12, 2018: Bone Marrow Transplantation
Nancy A Kernan, Paul G Richardson, Angela R Smith, Brandon M Triplett, Joseph H Antin, Leslie Lehmann, Yoav Messinger, Wei Liang, Robin Hume, William Tappe, Robert J Soiffer, Stephan A Grupp
BACKGROUND: Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is a potentially fatal complication of conditioning for hematopoietic stem cell transplantation (HSCT) but can occur after nontransplant-associated chemotherapy. Following HSCT, VOD/SOS with multi-organ dysfunction (MOD) may be associated with >80% mortality. Defibrotide is approved to treat severe hepatic VOD/SOS post-HSCT in patients aged >1 month in the European Union and hepatic VOD/SOS with renal or pulmonary dysfunction post-HSCT in the United States...
October 2018: Pediatric Blood & Cancer
Mutsumi Nishida, Kaoru Kahata, Eiko Hayase, Akio Shigematsu, Megumi Sato, Yusuke Kudo, Satomi Omotehara, Takahito Iwai, Junichi Sugita, Hitoshi Shibuya, Chikara Shimizu, Takanori Teshima
Sinusoidal obstruction syndrome (SOS)/hepatic veno-occlusive disease (VOD) is a well-documented complication after hematopoietic stem cell transplantation (HSCT). Transabdominal ultrasonography (US) enables the visualization of blood flow abnormalities and is therefore useful for the diagnosis of SOS/VOD. We herein prospectively evaluated accuracy of a novel US diagnostic scoring system of SOS/VOD based on US findings. We carried out US in 106 patients on day 14 and when SOS/VOD was suspected after allogeneic HSCT...
September 2018: Biology of Blood and Marrow Transplantation
Nancy A Kernan, Stephan Grupp, Angela R Smith, Sally Arai, Brandon Triplett, Joseph H Antin, Leslie Lehmann, Tsiporah Shore, Vincent T Ho, Nancy Bunin, Massimo Iacobelli, Wei Liang, Robin Hume, William Tappe, Robert Soiffer, Paul Richardson
Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is a potentially life-threatening complication of haematopoietic stem cell transplant (HSCT) conditioning and chemotherapy. Defibrotide is approved for treatment of hepatic VOD/SOS with pulmonary or renal dysfunction [i.e., multi-organ dysfunction (MOD)] after HSCT in the United States and severe VOD/SOS after HSCT in patients aged older than 1 month in the European Union. Defibrotide was available as an investigational drug by an expanded-access treatment programme (T-IND; NCT00628498)...
June 2018: British Journal of Haematology
Akshay Sharma, Guolian Kang, Anusha Sunkara, Hiroto Inaba, Sima Jeha, Shane J Cross, Terrence Geiger, Brandon Triplett
Recurrent/refractory hematologic malignancies have a poor prognosis, and there is a need for novel treatment regimens that can be tolerated by this heavily pretreated patient group. Clofarabine has antileukemic activity with an acceptable toxicity profile. In a phase I clinical trial (NCT00824135), we substituted clofarabine for fludarabine in a well-established reduced-intensity conditioning regimen for a T cell-depleted, mismatched-related (haploidentical) donor transplant backbone and explored the maximum tolerated dose of clofarabine in this combination in 15 patients undergoing hematopoietic cell transplantation for recurrent/refractory or secondary leukemia...
May 10, 2018: Journal of Pediatric Hematology/oncology
Samah Nassereddine, Saud Alsubait, Imad Tabbara
Hepatic sinusoidal obstruction syndrome (SOS) is a rare fatal clinical entity seen following hematopoietic stem cell transplant (HSCT). It is more commonly reported to occur following allogeneic HSCT compared to autologous HSCT. Historically, it is known as hepatitis following HSCT. It is thought that endothelial damage to the hepatic venules leading to occlusion of the terminal hepatic venules and hepatic sinusoids is the trigger for the development of SOS. Several risk factors have been associated with this condition...
May 2018: Anticancer Research
Ziyi Sun, Jianmei Kang, Yongchang Zhang
INTRODUCTION: Hepatic veno-occlusive disease (HVOD), as known as hepatic sinusoidal obstruction syndrome (HSOS), is an obliterative venulitis of the terminal hepatic venules, which is responsible for considerable mortality. The potential mechanism is destruction of hepatic sinusoidal endothelial cells (SEC), with sloughing and downstream occlusion of terminal hepatic venules. Here, we report a case of HVOD who have a history of ingestion of Gynura segetum for 1 month. The patient presents for abdominal pain and distension...
April 2018: Medicine (Baltimore)
Kelly J Norsworthy, Chia-Wen Ko, Jee Eun Lee, Jiang Liu, Christy S John, Donna Przepiorka, Ann T Farrell, Richard Pazdur
On September 2, 2017, the U.S. Food and Drug Administration approved gemtuzumab ozogamicin (GO; Mylotarg; Pfizer, New York City, NY) for treatment of relapsed or refractory (R/R) CD33-positive acute myeloid leukemia (AML) in patients 2 years of age and older. GO is a CD33-directed antibody drug conjugate linked to the cytotoxic antibiotic calicheamicin. It originally received accelerated approval for treatment of older patients with relapsed CD33-positive AML in 2000, but it was withdrawn from the market in 2010 when the confirmatory trial failed to demonstrate clinical benefit among safety concerns, such as a higher rate of induction fatalities on the GO combination arm compared with chemotherapy alone...
September 2018: Oncologist
H Hou, Y H Yao, J Lu, P F Xiao, X N Bian, H Liu, D X Hu, J Ling, J Li, Z Zhai, L J Kong, S Y Hu
Objective: To evaluate the efficiency and safety of low intensity conditional regimen for children with Fanconi anemia (FA) receiving allogenic hematopoietic stem cells transplantation (allo-HSCT). Methods: Four patients diagnosed as Fanconi anemia were enrolled in this study. One patient received HLA-identical sibling donor hematopoietic stem cell transplantation, two patients underwent unrelated donor matched (UD) HSCT, and one patient received unrelated cord blood transplantation. The conditional regimen consisted of Busulfan with low dose of cyclophosphamide...
March 14, 2018: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
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