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World J Hepatol 2022 May;14(5):1038-1046

Department of Gastroenterology and Hepatology, Institute for Clinical and Experimental Medicine, Prague 14021, Czech Republic.

Background: Transjugular intrahepatic portosystemic shunt (TIPS) is a method used to decrease portal hypertension. Biliary stricture is the rarest of the complications associated with this procedure with only 12 cases previously reported in the literature. None of these cases have documented the resolution of biliary stenosis induced by a stent graft. Read More

Front Immunol 2022 2;13:886540. Epub 2022 Jun 2.

Department of Pediatric Respiratory Medicine, Immunology and Intensive Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health (BIH), Berlin, Germany.

We report two patients with DNA repair disorders (Artemis deficiency, Ataxia telangiectasia) with destructive skin granulomas, presumably triggered by live-attenuated rubella vaccinations. Both patients showed reduced naïve T cells. Rapid resolution of skin lesions was observed following hematopoietic stem cell transplantation. Read More

Angiogenesis 2022 Jun 15. Epub 2022 Jun 15.

Mimetas, Leiden, The Netherlands.

With recent progress in modeling liver organogenesis and regeneration, the lack of vasculature is becoming the bottleneck in progressing our ability to model human hepatic tissues in vitro. Here, we introduce a platform for routine grafting of liver and other tissues on an in vitro grown microvascular bed. The platform consists of 64 microfluidic chips patterned underneath a 384-well microtiter plate. Read More

Int J Hematol 2022 May 30. Epub 2022 May 30.

Clinical Research Center, National Hospital Organization Nagoya Medical Center, Nagoya, Japan.

Inotuzumab ozogamicin (InO) is a CD22-directed antibody conjugated with calicheamicin approved for adult relapsed or refractory CD22-positive acute lymphoblastic leukemia (ALL). This phase 1 study primarily aimed to determine the pediatric recommended doses of InO through the standard 3 + 3 design, and to evaluate the safety, tolerability, pharmacokinetic (PK) profile, immunogenicity and efficacy of InO. Dose level 1 (DL1) was 1. Read More

Clin Drug Investig 2022 Jun 20;42(6):465-476. Epub 2022 May 20.

NOVEL Health Strategies, Bethesda, MD, USA.

Background And Objectives: Defibrotide is approved to treat severe veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) after haematopoietic cell transplantation in patients aged > 1 month in the European Union and for VOD/SOS with renal/pulmonary dysfunction post-haematopoietic cell transplantation in the United States. This meta-analysis estimated the incidence and risk of VOD/SOS after intravenous defibrotide prophylaxis using the published literature.

Methods: PubMed, Embase and Web of Science were searched through 30 November 2021 for defibrotide studies in VOD/SOS "prevention" or "prophylaxis," excluding phase I studies, case reports, studies with fewer than ten patients and reviews. Read More

Pulm Circ 2022 Apr 3;12(2):e12083. Epub 2022 May 3.

Department of Medicine, Pulmonary Hypertension Program King Faisal Specialist Hospital & Research Center Riyadh Saudi Arabia.

Even though pulmonary arterial hypertension (PAH) remains an incurable disease, the combination of PAH-specific therapies allowed evolving from symptom-based strategies to others aiming to move patients to low-risk conditions. Endothelin-1 (ET-1) receptor antagonists emerged as specific-PAH drugs that can be used in combination with other specific therapies. This work aimed to perform a prospective clinical assessment of patients with PAH that switched from bosentan to macitentan (POTENT), due to inadequate response. Read More

Rinsho Ketsueki 2022 ;63(4):271-276

Division of Hematology and Oncology, Department of Medicine, Kurume University School of Medicine.

Inotuzumab ozogamicin (InO) was administered in three cases of relapsed/refractory adult acute lymphoblastic leukemia (ALL) before allogeneic hematopoietic stem cell transplantation (allo-SCT). One case developed extremely severe sinusoidal obstruction syndrome (SOS) but recovered after receiving defibrotide therapy. A gap of 63 days in the SOS case was noted from the last administration of InO to allo-SCT, the duration was 133 and 86 days for the other two cases, and the remaining risk factors for SOS were comparable in the three cases. Read More

Chin J Nat Med 2022 Apr;20(4):270-281

The MOE Key Laboratory for Standardization of Chinese Medicines and the SATCM Key Laboratory for New Resources and Quality Evaluation of Chinese Medicines, Institute of Chinese Materia Medica, Shanghai University of Traditional Chinese Medicine, Shanghai 201203, China; Shanghai R & D Center for Standardization of Traditional Chinese Medicines, Shanghai 201203, China.

Hepatic sinusoidal obstruction syndrome (HSOS) via exposure to pyrrolizidine alkaloids (PAs) is with high mortality and there is no effective treatment in clinics. Bear bile powder (BBP) is a famous traditional animal drug for curing a variety of hepatobiliary diseases such as cholestasis, inflammation, and fibrosis. Here, we aim to evaluate the protective effect of BBP against HSOS induced by senecionine, a highly hepatotoxic PA compound. Read More

Mol Med Rep 2022 Jun 27;25(6). Epub 2022 Apr 27.

Department of Hepato‑Biliary‑Pancreatic Surgery, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa 920‑8641, Japan.

Sinusoidal obstruction syndrome (SOS) is a type of fatal hepatic injury, which predominantly occurs following exposure to drugs, such as oxaliplatin, or bone marrow transplantation. Extravasated platelet aggregation (EPA) plays an important role in the development of SOS in rat and mouse models. Furthermore, platelets invading the space of Disse adhere to hepatocytes and are phagocytized in patients with SOS. Read More

Curr Opin Gastroenterol 2022 05 4;38(3):221-229. Epub 2022 Mar 4.

Department of Medicine II, Medical Center University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.

Purpose Of Review: This review summarizes indications and contraindications for implantation of transjugular intrahepatic portosystemic shunt (TIPS). Further, patient selection strategies are discussed.

Recent Findings: TIPS implantation is a highly effective treatment for portal hypertension. Read More

Bull Natl Res Cent 2022 21;46(1):114. Epub 2022 Apr 21.

Acute Care Surgery Unit, Liverpool Hospital, 75 Elizabeth Street, Liverpool, NSW 2170 Australia.

Background: Vasculitis and phlebitis with vascular occlusion within appendix specimen is rare. Several authors have reported COVID-19 related veno-occlusive disease in hepatic pathology, but lymphoid aggregation with phlebitis is unusual in appendix specimen. We present a case with medium size venule phlebitis in an appendix of a patient recovered from COVID-19 infection. Read More

Zhonghua Xue Ye Xue Za Zhi 2022 Mar;43(3):177-183

Transplant Cell Ther 2022 04;28(4):179-180

The University of Texas Health Science Center at Houston, Houston, TX, USA. Electronic address:

Arch Toxicol 2022 Jul 31;96(7):2003-2019. Epub 2022 Mar 31.

The MOE Key Laboratory for Standardization of Chinese Medicines and the SATCM Key Laboratory for New Resources and Quality Evaluation of Chinese Medicines, Institute of Chinese Materia Medica, Shanghai University of Traditional Chinese Medicine, Shanghai, 201210, China.

Hepatic sinusoidal obstruction disease (HSOS) is a rare but life-threatening vascular liver disease. However, its underlying mechanism and molecular changes in HSOS are largely unknown, thus greatly hindering the development of its effective treatment. Hepatic sinusoidal endothelial cells (HSECs) are the primary and essential target for HSOS. Read More

Liver Transpl 2022 Jul 28;28(7):1257-1261. Epub 2022 Apr 28.

Hepatology and Liver Transplantation Unit, Hospital General Universitario Gregorio Marañón, Universidad Complutense, Madrid, Spain.

Zhonghua Er Ke Za Zhi 2022 Mar;60(3):246-247

Center for Pediatric Liver Diseases, Children's Hospital of Fudan University, Shanghai 201102, China.

Transpl Immunol 2022 04 24;71:101557. Epub 2022 Feb 24.

Department of Surgery, Division of Gastroenterological, General and Transplant Surgery, Jichi Medical University, Japan.

Background: Sinusoidal obstruction syndrome (SOS) after liver transplantation (LT) is a rare and potentially lethal complication. We retrospectively reviewed the outcomes of patients with post-transplant SOS.

Methods: Between May 2001 and December 2019, of 332 patients who underwent LT, 5 (1. Read More

J Pediatr Hematol Oncol 2022 Mar;44(2):e343-e348

Division of Pediatric Hematology, Oncology and Blood and Marrow Transplant.

Sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD) posthematopoietic stem cell transplantation (HSCT) is often diagnosed using the modified Seattle (MS) or European Society for Blood and Marrow Transplantation (EBMT) criteria. We hypothesized that strict application of these criteria could affect the timing of diagnosis and incidence of SOS/VOD. We collected data on 215 transplants performed in 184 patients at a single pediatric hematopoietic stem cell transplantation center, which were divided into 3 cohorts. Read More

Acta Cir Bras 2022 18;36(11):e361104. Epub 2022 Feb 18.

PhD. Faculty of Pharmacy - Zhejiang Pharmaceutical College - Ningbo, China.

Purpose: To investigate the underlying mechanism of hepatic sinusoidal obstruction syndrome (HSOS) induced by Gynura segetum by measuring autophagy in mouse models.

Methods: The model group was administered G. segetum (30 g/kg/d) by gavage, while the normal control group was administered an equal volume of saline daily for five weeks. Read More

Pathol Res Pract 2022 Mar 11;231:153799. Epub 2022 Feb 11.

Institute of Pathology, Germany; Tissue Biobank, University Medical Center Mainz, Langenbeckstr. 1, 55101 Mainz, Germany.

Pulmonary veno-occlusive disease (pVOD) is a potentially life-threatening sequela of allogeneic haematopoietic stem-cell transplantation (alloHSCT). We conducted a morphometric evaluation of autopsy lung tissue to determine the incidence of pVOD and its association with donor type, conditioning regime, hepatic sinusoidal obstruction syndrome (hSOS), survival time, and graft versus host disease (GvHD). The degree of occlusion of pulmonary veins in 78 autopsy cases after alloHSCT and 12 control cases was assigned to one of the following categories: none, minor thickening of the intima (up to 33% narrowing), moderate pVOD wherein about half of the lumen (34-66%) is occluded, or advanced pVOD with near total or total (67-100%) obliteration of the lumen. Read More

Bone Marrow Transplant 2022 05 17;57(5):705-711. Epub 2022 Feb 17.

University of Helsinki, Helsinki University Hospital, Children's Hospital, and Pediatric Research Center, Helsinki, Finland.

Treatment-related mortality and morbidity remain a challenge in hematopoietic stem cell transplantation (HSCT). In this retrospective, single-center study, we analyzed endothelial damage as a potential, common denominator and mechanism for the adverse effects. We evaluated the prevalence of key vascular complications and graft-versus-host disease among 122 pediatric patients with an allogeneic HSCT between 2001 and 2013. Read More

Indian J Radiol Imaging 2021 Oct 30;31(4):858-866. Epub 2021 Nov 30.

Department of Hepatology, Christian Medical College, Vellore, Tamil Nadu, India.

 The aim of the study is to evaluate the technical and clinical outcomes of transjugular intrahepatic portosystemic shunt (TIPS) performed with additional transabdominal ultrasound guidance.  Patients who underwent TIPS between January 2004 to January 2020 in our center were studied. Technical, hemodynamic, angiographic, and clinical outcome were recorded up to 1 year of follow-up. Read More

Transfus Apher Sci 2022 Feb 24;61(1):103369. Epub 2022 Jan 24.

Memorial Bahcelievler Hospital Adult Hematology and BMT Clinic, Istanbul, Turkey.

Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is one of the most life-threatening early complications following hematopoietic cell transplantation (HCT). Due to the high mortality rate of severe VOD/SOS accompanied with multiorgan failure, there is a great interest in preventive strategies. The efficacy of defibrotide (DF) on the prevention of VOD/SOS has been clearly shown in high-risk pediatric patients, but evidence-based data on adults is scarce. Read More

Bone Marrow Transplant 2022 04 3;57(4):664-667. Epub 2022 Feb 3.

Mother and Child Health, Santa Maria della Misericordia Hospital, Perugia, Italy.

Transfus Apher Sci 2022 Feb 22;61(1):103372. Epub 2022 Jan 22.

Yeditepe University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Istanbul, Turkey.

Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) affecting the liver is a rare, possibly life-threatening complication of hematopoietic stem cell transplantation (HSCT). Sinusoidal endothelial cell (SEC) damage triggered by various factors (especially conditioning regimen) results in post sinusoidal portal hypertension due to obstruction of the hepatic vein. Diagnosis is guided by traditional clinical diagnostic criteria; the modified Seattle criteria, the Baltimore and revised European Group for Blood and Marrow Transplantation (EBMT), specifically. Read More

Transfus Apher Sci 2022 Feb 24;61(1):103370. Epub 2022 Jan 24.

Yeditepe University Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Istanbul, Turkey.

Hepatic veno-occlusive disease (VOD), also termed sinusoidal obstruction syndrome (SOS), is a rare and life threatening complication of hematopoetic stem cell transplantation (HSCT). Many conditions can mimic the clinical signs of VOD/SOS. Differential diagnosis and diagnosis of the disease at an early stage is important, since the severe form of the disease has higher mortality rates and early-initiated specific treatment has better response rates. Read More

Pediatr Transplant 2022 06 26;26(4):e14229. Epub 2022 Jan 26.

Pediatric Neuro-Oncology, Division of Pediatric Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.

Background: Medulloblastoma outcomes have improved with craniospinal irradiation and chemotherapy, but such therapy has resulted in poor neurocognitive outcomes for young patients. Chemotherapy-only regimens with autologous transplant have been implemented with the intention of avoiding radiation. It is not yet known whether single or tandem transplantation is superior with respect to efficacy and/or safety. Read More

Bone Marrow Transplant 2022 04 24;57(4):538-546. Epub 2022 Jan 24.

Department of Hematology, Catholic Hematology Hospital and Leukemia Research Institute, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea.

Using traditional statistical methods, we previously analyzed the risk factors and treatment outcomes of veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) after allogeneic hematopoietic cell transplantation. Within the same cohort, we applied machine learning to create prediction and recommendation models. We analyzed 2572 transplants using eXtreme Gradient Boosting (XGBoost) to predict post-transplant VOD/SOS and early death. Read More

Transplant Cell Ther 2022 04 19;28(4):196-202. Epub 2022 Jan 19.

Department of Clinical Pharmacy, University Medical Center Utrecht/Wilhelmina Children's Hospital, Utrecht, the Netherlands.

Intravenous busulfan is widely used as part of myeloablative conditioning regimens in children and young adults undergoing allogeneic hematopoietic cell transplantation (HCT). Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is a serious clinical problem observed with busulfan-based conditioning HCT. The development of VOD/SOS may be associated with busulfan exposure. Read More

Eur J Case Rep Intern Med 2021 17;8(12):003081. Epub 2021 Dec 17.

Internal Medicine, Rochester General Hospital, Rochester, New York, USA.

Budd-Chiari syndrome (BCS) results from an occlusion of the hepatic venous flow which in turn leads to portal hypertension causing ascites and other signs of liver dysfunction. Here, we present the case of a 43-year-old woman with recurrent ascites who was found to have BCS secondary to an inferior vena cava thrombosis extending into the hepatic veins. Although she had a normal platelet count on admission, additional laboratory investigations revealed an MPL mutation. Read More