Pdf hepatic venoocclusive disease antonio ruggiero. Strategies for the management of hepatic venoocclusive. In the western hemisphere, vod, classified as a conditioningrelated toxicity, is most commonly caused. Vod can range from mild to severe, and occurs in approximately 10 30% of childrenyoung people who have received total body irradiation tbi, busulfan, or high dose melphalan as part of their conditioning treatment before a stem cell transplant. Hepatic veno occlusive disease is a condition that blocks occludes small veins in the liver, disrupting blood flow in this organ. Clinical signs and symptoms of venoocclusive disease include hepatomegaly, right upper quadrant pain, ascites, fluid retention, weight gain, and hyperbilirubinemia, usually developing by 30 days after transplantation. Sep 17, 20 a joint working group established by the haemato. Incidence of anicteric venoocclusive diseasesinusoidal.
Hepatic venoocclusive disease radiology reference article. Pulmonary venoocclusive disease is a rare and usually fatal condition in which there is gradual obliteration of the pulmonary veins and venules. Several clinical publications have confirmed that administration of. This causes a decrease in blood flow inside the liver and may lead to liver damage. Hepatic sinusoidal obstruction syndrome sos, previously termed hepatic venoocclusive disease vod, is one of the most feared complications of allogeneic and autologous hematopoietic cell. Treatment of severe hepatic venoocclusive disease after bone. Hepatic veno occlusive disease vod, increasingly referred to as sinusoidal obstruction syndrome, is a wellrecognized complication of hematopoietic stem cell transplantation and contributes to considerable morbidity and mortality.
Hepatic venoocclusive disease vod, also known as sinusoidal obstruction syndrome sos, can develop in a subset of patients, primarily after myeloablative hematopoietic stem cell transplantation, but it also may occur after reducedintensity conditioning. See jamiacan vomiting sickness lung see pulmonary veno occlusive disease. Sinusoidal obstruction syndrome or venoocclusive disease sosvod is a potentially lifethreatening complication of hematopoietic sct. Hepatic sinusoidal obstruction syndrome sos is an obliterative venulitis of the terminal hepatic venules, which in its more severe forms imparts a high risk of mortality. Oct 12, 2019 hepatic veno occlusive disease is a pathological condition in which there is blockage of minute veins in the liver. Pulmonary venoocclusive disease pvod is a rare form of pulmonary hypertension caused by progressive blockage of the small veins in the lungs. Pooled analysis of day 100 survival for defibrotide. Busulfan and venoocclusive disease of the liver annals. Pooled results showed that, compared with the mean bu auc i. Venoocclusive hepatic disease clinical presentation. Venoocclusive disease is not another illness, but a complication.
Although pulmonary venoocclusive disease is a rare condition, its incidence is apparently on the increase. Venoocclusive disease of the liver vod is an uncommon, early complication of hematopoietic cell transplantation hct that is associated with significant mortality 1,2. Hepatic venoocclusive diseasesinusoidal obstruction syndrome vodsos is a potentially lifethreatening complication of hematopoietic cell transplantation hct. Hepatic venoocclusive disease sinusoidal obstruction. Intravenous defibrotide was administered in doses ranging from 5 mgkg per day to 60 mgkg per day. A left lung transplantation was performed successfully and pathologic examination of the explanted lung showed severe changes compatible with vod. Prophylactic, preemptive, and curative treatment for. Revised diagnosis and severity criteria for sinusoidal obstruction. Apr 09, 2018 the outlook for hepatic veno occlusive disease depends on the causative factor of the condition. Abstract pulmonary veno occlusive disease pvod is a rare form of pulmonary hypertension ph characterised by preferential remodelling of the pulmonary venules. Venoocclusive disease of the liver after lung transplantation. Veno occlusive disease, also known as sinusoidal obstruction syndrome vodsos, is a potentially lifethreatening complication of allogeneic or autologous hematopoietic stem cell transplantation. These criteria predict venoocclusive disease with an accuracy of more than 90% but have a relatively low sensitivity of 56%. Sarcoidosisrelated pulmonary venoocclusive disease.
Sinusoidal obstruction syndrome, also known as veno occlusive disease sosvod, is a potentially lifethreatening complication that can develop after hematopoietic cell transplantation hct. Published reports of venoocclusive disease vod, also widely referred to as sinusoidal obstruction syndrome sos, first appeared. Jun 18, 2018 veno occlusive disease of the liver vod is an uncommon, early complication of hematopoietic cell transplantation hct that is associated with significant mortality 1,2. It has a reported overall mean incidence of 814%, with an incidence of up to 60% in some highrisk populations 1, 2, 3. Recent developments with defibrotide for the treatment of hepatic venoocclusive diseasesinusoidal obstruction syndrome christine duncan a, justine kahnb, stephan a. The blockage leads to high blood pressures in the arteries of the lungs, which, in turn, leads to heart failure. Veno occlusive disease of the liver after lung transplantation. Hepatic venoocclusive disease vod, also known as sinusoidal obstruction syndrome sos, can develop in. Hepatic venoocclusive disease can occur at any age. Hepatic venoocclusive disease genetic and rare diseases. Phase 3 trial of defibrotide for the treatment of severe veno. Pulmonary venoocclusive disease genetic and rare diseases. It accounts for a significant fraction of transplantrelated mortality and, in its severe form, is almost always fatal. Venoocclusive disease vod sinusoidal obstructive syndrome sos, also called hepatic venoocclusive disease vod, is part of a spectrum of organ injury syndromes that occur after hsct.
Manifestations and management of venoocclusive diseasesinusoidal obstruction syndrome in the era of contemporary therapies priti tewari, md, whitney wallis, pharmd, and partow kebriaei, md dr. Clinical presentation right upper quadrant pain painful hepatomegaly ascites abnormal liver. She gave a 3yr history of dry cough, episodic wheeze and mild exertional dyspnoea. Busulfan systemic exposure and its relationship with. Hepatic venoocclusive disease vod, also called sinusoidal obstruction syndrome sos, is a potentially fatal complication of allogeneic or autologous hematopoietic stem cell transplantation. Hepatic venoocclusive disease an overview sciencedirect. Hepatic veno occlusive disease vod is a clinical syndrome characterized by weight gain fluid, tender hepatomegaly, and elevations of hepatocellular enzymes and bilirubin. Familial hepatic venoocclusive disease with immunodeficiency vodi, omim. Venoocclusive disease definition of venoocclusive disease. Sos, also known as veno occlusive disease vod, occurs as a result of cytoreductive. Hepatic venoocclusive disease after hematopoietic stem cell.
Clinical diagnosis of venoocclusive disease using contrast. Venoocclusive disease vod, also known as sinusoidal obstruction syndrome, is a potentially lifethreatening complication of hsct the conditioning regimens given before hsct result in the. Compositae is a well recognized medicinal plant in indonesia and malaysia. Richardson et al reported on 19 patients who had received defibrotide for the management of severe veno occlusive disease in a compassionate use study richardson et al, 1998. Mar 23, 2015 sinusoidal obstruction syndrome or veno occlusive disease sosvod is a potentially lifethreatening complication of hematopoietic sct hsct. Hepatic sinusoidal obstruction syndrome sos, previously termed hepatic veno occlusive disease vod, is one of the most feared complications of allogeneic and autologous hematopoietic cell transplantation hct. In developed countries, hepatic veno occlusive disease is mainly associated with the conditioning regimen for hematopoietic stem cell transplantation, with 10 to 60% of transplant patients depending on the conditioning regimen protocol used developing hepatic vod. She gave a 3yr history of dry cough, episodic wheeze and. Sinusoidal obstruction syndrome, also known as venoocclusive disease sos vod, is a potentially lifethreatening complication that can develop after hematopoietic cell transplantation hct. Abstract pulmonary venoocclusive disease pvod is a rare form of pulmonary hypertension ph characterised by preferential remodelling of the pulmonary venules. Longo, in critical care medicine third edition, 2008. Hepatic venoocclusive disease vod, increasingly referred to as sinusoidal obstruction syndrome, is a wellrecognized complication of hematopoietic stem cell transplantation and contributes to. Severe vodsos, typically characterized by multiorgan failure, has been associated with a mortality rate greater than 80%. In severe cases, multiple organ failure or severe bacterial infections may occur.
Hepatic venoocclusive disease, or sinusoidal obstruction syndrome, is a common and potentially fatal complication following hsct. Manifestations and management of venoocclusive disease. Mar 31, 2016 hepatic veno occlusive disease vod, also called sinusoidal obstruction syndrome sos, is a potentially fatal complication of allogeneic or autologous hematopoietic stem cell transplantation hsct. Venoocclusive disease vod is one of the less common but still serious complications which can occur during stem cell transplant sct. Risk score for the development of venoocclusive disease.
Without a lung biopsy the conical diagnosis of this. Phase 3 trial of defibrotide for the treatment of severe. Dec 12, 2015 assess the efficacy and safety outcomes associated with the use of traditional and novel therapies for the management of hepatic venoocclusive disease describe how novel agents for the prevention and treatment of hepatic venoocclusive disease can be effectively incorporated into clinical practice. Sinusoidal obstruction syndrome or venoocclusive disease sosvod is a potentially lifethreatening complication of hematopoietic sct hsct. Hepatic veno occlusive disease hvod caused by gynura segetum has been increasingly reported in china in recent years. This factsheet has been produced to help you understand more about vod. Risk factors for development of and progression of hepatic veno. The principal cause of most cases of venoocclusive disease is the toxicity of the preparative regimen for bmt.
Thenature ofthis toxin is unknown, but the frequent association of. Pulmonary venoocclusive disease pvod is a rare and highly lethal disorder of the pulmonary vasculature. Rare, usually children and adolescents, especially females also after cancer chemotherapy causes pulmonary hypertension secondary to widespread thromboemboli of large and medium size branches of pulmonary vein. Mar 01, 2020 a collection of disease information resources and questions answered by our genetic and rare diseases information specialists for pulmonary venoocclusive disease.
People undergoing radiation therapy for treatment of some form of cancer are also at risk for developing hepatic veno occlusive disease. When linked to hematopoietic transplant, over thirty percent cases prove to be fatal with a high mortality rate of the severe cases. Highdose busulfan in combination therapy is well known to be responsible for venoocclusive disease of the liver in about 20% of adult patients undergoing bone marrow. Venoocclusive disease vod, also known as sinusoidal obstruction syndrome, is a potentially lifethreatening complication of hsct the conditioning regimens given before hsct result in the production of toxic metabolites by the hepatocytes in the liver these metabolites trigger the activation, damage and inflammation of the endothelial.
The patient denied fever, sweats or weight loss and had no significant past medical. According to the modified seattle criteria, 2 or more of the following must be. It is a complication of highdose chemotherapy given before a bone marrow transplant bmt and is marked by weight gain due to fluid retention, increased liver size, and raised levels of bilirubin in the blood. Venoocclusive disease, also known as sinusoidal obstruction syndrome vodsos, is a potentially lifethreatening complication of allogeneic or autologous hematopoietic stem cell transplantation. Hepatic sinusoidal obstruction syndrome sos, previously termed hepatic veno occlusive disease vod, is characterized by hepatomegaly, right upper quadrant pain, jaundice, and ascites, most often occurring in patients undergoing hematopoietic cell transplantation hct and less commonly following the use of certain chemotherapeutic agents in nontransplant settings, ingestion. Hepatic veno occlusive disease vod, also known as sinusoidal obstruction syndrome sos, is a condition arising from occlusion of hepatic venules. Vod has been described in a patient who drank an infusion of a traditional herbal medicine that contained pyrrolizidine alkaloids. Sinusoidal obstruction syndrome, also known as venoocclusive disease sosvod, is a potentially lifethreatening complication that can develop after hematopoietic cell transplantation hct. Recent developments with defibrotide for the treatment of. In developed countries, hepatic venoocclusive disease is mainly associated with the conditioning.
Venoocclusive disease vod, also known as hepatic sinusoidal obstruction syndrome sos, defines a disease based on an incomplete obstruction of sinusoids and venules with the. Rare, usually children and adolescents, especially females also after cancer chemotherapy causes pulmonary hypertension secondary to widespread thromboemboli of large and medium size. Venoocclusive disease, also known as sinusoidal obstruction syndrome vodsos, is a potentially lifethreaten. Hepatic venoocclusive disease vod is a complication after hematopoietic stem cell. Unique mutation in sp110 resulting in hepatic veno. Nov 01, 2008 in severe cases, multiple organ failure or severe bacterial infections may occur. Pulmonary veno occlusive disease pvod is a rare form of pulmonary hypertension caused by progressive blockage of the small veins in the lungs. Thirteen studies involving 548 pediatric patients aged 0. Assess the efficacy and safety outcomes associated with the use of traditional and novel therapies for the management of hepatic venoocclusive disease describe how novel agents for the. Hepatic venoocclusive disease with immunodeficiency nih. Definition of hepatic venoocclusive disease nci dictionary.
Hepatic venoocclusive disease vod is a clinical syndrome characterized by hepatomegaly, ascites, weight gain and jaundice. Hepatic venoocclusive disease violetta kivovich md, phd 2. Pdf diagnosis and severity criteria for sinusoidal obstruction. Hepatic venoocclusive disease vod, also known as sinusoidal obstruction syndrome sos, can develop in a subset of patients, primarily after myeloablative hematopoietic stem cell transplantation. The patient developed severe pulmonary hypertension and cor pulmonale. Hepatic venoocclusive disease after hematopoietic stem. Hepatic venoocclusive disease is a condition that blocks occludes small veins in the liver, disrupting blood flow in this organ. Sinusoidal obstruction syndrome hepatic venoocclusive. Hepatic veno occlusive disease can occur at any age.
Venoocclusive disease is not another illness, but a complication that affects the liver. Hepatic veno occlusive disease or veno occlusive disease with immunodeficiency vodi is a condition in which some of the small veins in the liver are obstructed. Its signs and symptoms appear after the first few months of life. Sinusoidal obstruction syndrome, also known as venoocclusive disease sos vod, is a potentially life threatening complication that can. Sos usually affects 10% to 30% of patients after allogeneic stem cell transplantation sousa, 2012.
Sinusoidal obstruction syndrome hepatic venoocclusive disease. Pulmonary venoocclusive disease pvod is a rare disorder that can be misdiagnosed as idiopathic pulmonary arterial hypertension pah and accounts for 510% of cases initially considered as. Hepatic venoocclusive disease sinusoidal obstruction syndrome. Diagnosis and severity criteria for sinusoidal obstruction. Venoocclusive disease vod, also known as hepatic sinusoidal obstruction syndrome sos, defines a disease based on an incomplete obstruction of sinusoids and. Hepatic venoocclusive disorder or hepatic vod in a medical condition in which the sinusoidal capillaries present within the liver undergo injury due to consumption of toxic entities like. Pulmonary venoocclusive disease pvod is characterized by the blockage occlusion of the blood vessels that carry oxygenrich oxygenated blood from the lungs to the heart the pulmonary veins. Pulmonary veno occlusive disease pvod is a rare and highly lethal disorder of the pulmonary vasculature. This factsheet has been written to help you, as a young person, parent or carer to understand venoocclusive disease vod one of the less common but still serious complications which can occur during stem cell transplant sct. Before 1960, pulmonary venoocclusive disease was virtually unknown, though two cases from that period are now generally accepted as such. A 52yrold female nonsmoker presented with haemoptysis consisting of streaks of bright red blood daily for 3 weeks.
Venoocclusive disease is not another illness, but a. Veno occlusive disease may also be referred to as sinusoidal obstruction syndrome sos. In contrast to the insights into pathophysiology and management that have characterized. Hepatic venoocclusive disease vod, increasingly re ferred to as sinusoidal obstruction syndrome, is a well recognized complication of hematopoietic stem. What is hepatic venoocclusive diseasecausessymptoms. Sinusoidal obstruction syndromevenoocclusive disease. Hepatic veno occlusive disease with immunodeficiency also called vodi is a hereditary disorder of the liver and immune system. Veno occlusive disease is not another illness, but a complication that affects the liver. Hepatic venoocclusive disease with immunodeficiency also called vodi is a hereditary disorder of the liver and immune system. The symptoms had not improved with inhaled corticosteroids fluticasone 250. Hepatic sinusoidal obstruction syndromevenoocclusive disease sosvod is a lifethreatening complication of hct that belongs to a group. Hepatic veno occlusive disease vod, also known as sinusoidal obstruction syndrome sos, can develop in a subset of patients, primarily after myeloablative hematopoietic stem cell transplantation, but it also may occur after reducedintensity conditioning.
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