Pulmonary venoocclusive disease multimedia encyclopedia. Pathology of pulmonary venoocclusive disease and pulmonary. Pathology outlines sinusoidal obstruction syndrome. Its signs and symptoms appear after the first few months of life. Pulmonary venoocclusive disease radiology reference. What is hepatic venoocclusive diseasecausessymptoms. In veno occlusive disease vod is an uncommon liver problem in which the liver cannot function properly. The pathology, diagnosis, and treatment of hepatic veno. This chapter focuses on hepatic venoocclusive disease with immunodeficiency vodi, including the historic and scientific background, clinical. Veno occlusive disease of the liver after marrow transplantation. Venous and arterial changes in pulmonary venoocclusive. Sinusoidal obstruction syndrome or hepatic venoocclusive disease is a rare disease characterized by hepatic sinusoid.
Hepatic venoocclusive disease an overview sciencedirect. 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. Liver histology demonstrates obstruction of sinusoids in central areas with hepatocyte necrosis and hemorrhage. Venoocclusive disease vod, more recently called sinusoidal obstruction syndrome, is a group of disorders that mimic bcs, but it involves distinctly different populations of patients and is precipitated by distinctly different circumstances. Veno occlusive disease of the liver is a significant and often lifethreatening complication of marrow or stem cell transplantation. Veno occlusive disease sinusoidal obstruction syndrome veno occlusive disease is defined by the presence of prominent obstructive nonthrombotic lesions of the small hepatic veins in individuals exposed to either radiation or a hepatotoxin. The table below gives a brief summary of graft complications. This chapter focuses on hepatic veno occlusive disease with immunodeficiency vodi, including the historic and scientific background, clinical. It accounts for a significant fraction of transplantrelated mortality and, in its severe form, is almost always fatal.
Veno occlusive disease of the liver after allogeneic bone marrow transplantation in children with hematologic malignancies. Granulomas may also be related to other exogenous factors such as inhaled nonrefractile foreign material or aspirated food particles, or to endogenous deposition of amyloid or hemosiderin as seen in hemosiderosis or pulmonary veno occlusive disease. Jul 03, 20 hepatic veno occlusive disease with immunodeficiency vodi is characterized by 1 primary immunodeficiency and 2 terminal hepatic lobular vascular occlusion and hepatic fibrosis manifest as hepatomegaly andor hepatic failure. When linked to hematopoietic transplant, over thirty percent cases prove to be fatal with a high mortality rate of the severe cases. Sos, also referred to as veno occlusive disease, is a rare and highly lethal condition of the liver due to vascular injury. Hepatic venoocclusive disease hepatic vod is a condition resulting from toxic injury to the hepatic sinusoidal capillaries that leads to obstruction of the small hepatic veins. Hepatic venoocclusive disease genetic and rare diseases. Pvod has been described as idiopathic or complicating other conditions, including connective tissue diseases, hiv infection, bone marrow transplantation, sarcoidosis. Some diseases are acute, producing severe symptoms that terminate after a short time, e. Hepatic venoocclusive disease is a condition that blocks occludes small veins in the liver, disrupting blood flow in this organ. Pdf venoocclusive disease vod of the liver in cheetah. Sinusoidal obstruction syndrome, also known as venoocclusive disease sos vod, is a potentially lifethreatening complication that can. Hepatic veno occlusive disease vod is one of the most common and important regimenrelated toxicities observed after hematopoietic stem cell transplantation hsct. Pulmonary vaso occlusive disease pulmonary veno occlusive disease pvod is a very rare disease.
Hepatic venoocclusive disease vod, also known as sinusoidal obstruction syndrome sos, is a condition arising from occlusion of hepatic venules. Now it is considered an important cause of non cirrhotic portal hypertension particularly in children. The incidence and etiology of mivod remain unclear. Vod is defibrotide,84,85 a novel polydeoxyribonucleotide with adenosine. See jamiacan vomiting sickness lung see pulmonary venoocclusive disease. Venoocclusive disease after a blood and marrow transplant. Pathology of noncirrhotic liver disease request pdf. Sinusoidal obstruction syndrome venoocclusive disease. Pathological diagnosis of granulomatous lung diseases dr. The immunodeficiency comprises severe hypogammaglobulinemia, clinical evidence of tcell. Imaging description pulmonary venoocclusive disease pvod is considered a cause of pulmonary hypertension that preferentially affects the postcapillary pulmonary vasculature. See jamiacan vomiting sickness lung see pulmonary veno occlusive disease.
Hepatic venoocclusive disease vod is one of the most serious complications following hematopoietic stem cell. Learn about veno occlusive disease, which is an uncommon complication after blood and marrow transplant. Hepatic venoocclusive disease with immunodeficiency also called vodi is a hereditary disorder of the liver and immune system. Oct 16, 2018 pulmonary veno occlusive disease after chemotherapy. Hepatic veno occlusive disease vod, also known as sinusoidal obstruction syndrome sos, is a condition arising from occlusion of hepatic venules. It complicates up to 70% of hematopoietic cell transplantation and is associated with high morbidity and mortality rates.
In contrast to bcs, vod occurs in the sublobular branches of the hepatic veins, the terminal hepatic. The pathologic hallmark of pvod is the extensive and diffuse occlusion of pulmonary veins by fibrous tissue. Hematopoietic stem cell transplantation hsct is most often implicated, but sos is also associated with drug toxicity. Pulmonary veno occlusive disease after chemotherapy. Hepatic venoocclusive disease vod is a clinical syndrome characterized by weight gain fluid, tender hepatomegaly, and elevations of hepatocellular enzymes and bilirubin. The veno occlusive disease of the liver vod is a disorder caused by the nonthrombotic occlusion of the central veins of hepatic lobules.
The pathophysiology of veno occlusive disease culminates in occlusion of the pulmonary blood vessels. This causes a decrease in blood flow inside the liver and may lead to liver damage. Hepatic venoocclusive disease immunodeficiency syndrome is characterized by the association of severe hypogammaglobulinemia, combined t and b cell immunodeficiency, absent lymph node germinal centers, absent tissue plasma cells and hepatic venoocclusive disease. Hepatic venoocclusive disease radiology reference article.
This chapter focuses on hepatic venoocclusive disease with immunodeficiency vodi, including the historic and scientific. Hepatic venoocclusive disease with immunodeficiency oxford. The lancet original articles veno occlusive disease of the liver essential pathology g. Pdf in a retrospective study of autopsy material, 2 cheetahs and 3 snowleopards showed occlusive lesions of the centrilobular and sublobular hepatic. Venoocclusive disease definition of venoocclusive disease. Please be advised that we experienced an unexpected issue that occurred on saturday and sunday january 20th and 21st that caused the site to be down for an extended period of time and affected the ability of users to access content on wiley online library.
This was characterized by hyperbilirubinemia peaking at greater than or equal to 2 mgdl with at least 2 of 3 other findings. The clinical syndrome of hepatic venoocclusive disease vod after hematopoietic stem cell transplantation sct is characterized by liver enlargement and pain, fluid retention, weight gain and jaundice 1, 2, 3. The laboratory workup of a patient with possible veno occlusive disease has several objectives. Longo, in critical care medicine third edition, 2008. Clinical presentation right upper quadrant pain painful hepatomegaly ascites abnormal liver. You or someone you know may have been diagnosed with a retinal vein occlusion. A persistently elevated aspartate aminotransferase sgot prior to transplant was associated. Hepatic venoocclusive disease vod is a clinical syndrome characterized by weight gain fluid, tender hepatomegaly, and elevations of. Clinical signs and symptoms of veno occlusive disease. Definition of hepatic venoocclusive disease nci dictionary.
Prevalence is unknown but hepatic vod is a rare disease. 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. 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. Hepatic venoocclusive disease vod as a distinct clinical entity was first. These lung arteries are directly connected to the right side of the heart. Sos can present in an acute, subacute or chronic form usually with abdominal pain and swelling, with evidence of portal hypertension and variable degrees of serum. Vod can occur in as high as 70% of patients and is the most common hepatic complication in the immediate posttransplant period. Wanless, in surgical pathology of the gi tract, liver, biliary tract, and pancreas second edition, 2009. Hepatic veno occlusive disease, or sinusoidal obstruction syndrome, is a common and potentially fatal complication following hsct. Farber cancer institute, brigham and womens hospital, and the childrens hospital, harvard medical school, boston, massachusetts, u. Venoocclusive disease of the liver after allogeneic bone.
The first goal, of course, is to confirm the diagnosis, the second goal is to look for a detectable discrete cause, and the third goal is to establish the function of the liver and other end organs. Venoocclusive disease of the liver in captive cheetah s. Pulmonary venoocclusive disease pvod is currently classified as a subgroup of pulmonary arterial hypertension pah and accounts for 510% of cases initially considered to be idiopathic pah. The blockage leads to high blood pressures in the arteries of the lungs, which, in turn, leads to heart failure. Pulmonary veno occlusive disease pvod is a very rare disease. The syndrome is due to endothelial toxicity from highdose chemotherapy and results in a local. The imaging findings are a result of this fibrotic occlusion. Prophylactic, preemptive, and curative treatment for sinusoidal. Sos can present in an acute, subacute or chronic form usually with abdominal pain and swelling, with evidence of portal hypertension and variable degrees of. Pulmonary veno occlusive disease pvod is a rare cause of pulmonary hypertension ph. 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. Pulmonary venoocclusive disease european respiratory.
Hepatic venoocclusive disease or veno occlusive disease with immunodeficiency vodi is a condition in which some of the small veins in the liver are obstructed. In most cases, affected individuals experience recurrent infections, but they may also suffer from autoimmune diseases and malignancies. Successful treatment of hepatic venoocclusive disease. Hepatic venoocclusive disease with immunodeficiency vodi is characterized by 1 primary immunodeficiency and 2 terminal hepatic lobular vascular occlusion and hepatic fibrosis manifest as hepatomegaly andor hepatic failure. The term veno occlusive disease of the liver refers to a form of toxic liver injury characterized clinically by the development of hepatomegaly, ascites, and jaundice, and histologically by diffuse damage in the centrilobular zone of the liver. Previously called venoocclusive disease semin liver dis 2002. Developed by renowned radiologists in each specialty, statdx provides comprehensive decision support you can rely on venoocclusive disease. Hepatic venoocclusive disease with immunodeficiency. Pulmonary veno occlusive disease pulmonary vaso occlusive disease. Pvod 30pch 30 pvod is a rare pulmonary vascular disease causing ph. The pathology, diagnosis, and treatment of hepatic venoocclusive disease.
Buddchiari syndrome and venoocclusive disease clinical gate. Mivod is difficult to diagnose because of its rarity and frequent confusion with other diseases. Lager veins and those affected by buddchiari syndrome are spared the term veno occlusive disease of the liver refers to a form of toxic liver injury characterized clinically by the development of hepatomegaly, ascites, and jaundice, and. Pulmonary veno occlusive disease pathology tunica intima pathology tunica media pathology. Liver venoocclusive disease an overview sciencedirect topics. Venous and arterial changes in pulmonary veno occlusive disease, mitral stenosis and fibrosing mediastinitis. Pulmonary veno occlusive disease in an adult following bone marrow transplantation.
Pulmonary veno occlusive disease may have a genetic basis, published reports have indicated fatal occurrences that appeared to possess a familial pattern, more to the point, a germline mutation. Sinusoidal obstruction syndrome venoocclusive disease ncbi. 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. Veno occlusive disease of the liver is a less frequent but severe adverse effect. Successful surgical management of mesenteric inflammatory.
The first goal, of course, is to confirm the diagnosis, the second goal is to look for a detectable discrete cause, and the third goal is. Professor of pathology university college of the west indies, jamaica several diseases of the liver, though of different pathogenesis, possibly have a common factore. Sinusoidal obstruction syndrome pathology outlines. The clinical features are similar to those of intrahepatic portal hypertension unexplained weight gain, ascites, painful hepatomegaly, jaundice. Pulmonary venoocclusive disease pvod is a rare form of pulmonary hypertension caused by progressive blockage of the small veins in the lungs. Rollins 1989, stated that hvod is a nonthrombotic obliteration of small intrahepatic veins by loose connective tissues. Misdiagnosis of the disease is common since pvod presents with clinical and radiographic features mimicking idiopathic pulmonary arterial hypertension or even ph due to interstitial lung disease. 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. Vod is a classic complication of chemoirradiation therapy prior to bone marrow transplantation 2 and is also thought to be related to. Noninvasive imaging diagnosis of sinusoidal obstruction. Venoocclusive disease in snow leopards panthera uncia from. Sinusoidal obstruction syndrome sos, previously known as veno occlusive disease vod, is a distinctive and potentially fatal form of hepatic injury that occurs predominantly, if not only, after drug or toxin exposure.
The pathology of noncirrhotic liver diseases causing portal hypertension such as extrahepatic portal obstruction, idiopathic portal hypertension, nodular regenerative hyperplasia, veno occlusive. Pulmonary veno occlusive disease generally carries a poor prognosis some report a 72% mortality at one year from diagnosis 1. A phase ii study points towards a percentage of overall hematologic response around 30% in the setting of. Hepatic vod consists of occlusive fibrosis of small intrahepatc veins. Hepatic veno occlusive disease with immunodeficiency also called vodi is a hereditary disorder of the liver and immune system. Immunohistochemical identification of the material within occluded central venules.
Hepatic venoocclusive disease after hematopoietic stem cell. The preseptal and septal venules are affected and become fibrotic and thickened due to cellular proliferation. The disease is progressive and fatal, with median survival of about 2 years from the time of diagnosis to death. Nov 01, 2008 in severe cases, multiple organ failure or severe bacterial infections may occur. It leads to high blood pressure in the lung arteries pulmonary hypertension. Veno occlusive disease, which developed in 54% of bone marrow transplant recipients, is frequently associated with renal and cardiopulmonary failure.
Endemic zones have been identified in which the disease is associated with the consumption of. Although blood and marrow transplants can save patients lives, they can also result in numerous complications, including infections, renal failure, and liver complications, such as venoocclusive disease vod. The outlook for hepatic venoocclusive disease depends on the causative factor of the condition. The incidence of vod after hematopoietic cell transplantation bone marrow, peripheral blood progenitor cells or cord blood cells ranges from 5% to more than 60% in children and is similar in adults. Mar 31, 2016 the laboratory workup of a patient with possible veno occlusive disease has several objectives. The pattern and prevalence of veno occlusive disease in these leopards was similar to that reported in captive cheetah acinonyx jubatus, suggesting that a common extrinsic factor may cause the majority of hepatic disease in these large felid animals in captivity. Symptoms of vod include jaundice, pain or tenderness in the liver, rapid weight gain, edema, and ascites. The term mesenteric inflammatory veno occlusive disease mivod is used to describe an ischemic injury resulting from phlebitis or venulitis that affects the bowel or mesentery in the absence of arteritis.
Venoocclusive disease of the liver in renal transplant patients. Graftvshost disease gvhd is a donor tcellmediated disorder affecting the recipients skin, gastrointestinal tract, lungs, and liver. Hepatic veno occlusive disease is a condition that blocks occludes small veins in the liver, disrupting blood flow in this organ. The main hepatic lesion, a vascular lesion resembling veno occlusive disease vod of the liver and characterized by subendothelial fibrosis and proliferation of smooth musclelike cells in the central veins, was seen in 60% of the sexually mature cheetah. Venoocclusive disease of the liver and multiorgan failure. Primary immunodeficiency diseases are inherited disorders that affect human adaptive and innate immunity. We have prepared the following explanation to help you understand this condition better. Hepatic venoocclusive disease sinusoidal obstruction syndrome.
Pulmonary venoocclusive disease pvod is a very rare disease. Since diagnosis is based on clinical criteria, the incidence reported and severity. Hepatic venoocclusive disease violetta kivovich md, phd 2. Hepatic veno occlusive disease after bone marrow transplantation. Hepatic venoocclusive disease an overview sciencedirect topics. Veno occlusive disease vod of the liver is characterized by histological findings which include fibrous obliteration of the small hepatic veins by connective tissue and centrilobular necrosis. Pretransplant transaminase elevations, use of highdose cytoreductive therapy, and persistent fever during cytoreductive therapy are independent predictors of severe vod. Severe vodsos, typically characterized by multiorgan failure, has been associated with a mortality rate greater than 80%. The high blood pressure occurs in the pulmonary arteries. The immunodeficiency comprises severe hypogammaglobulinemia, clinical evidence of tcell immunodeficiency with normal numbers.