Veno occlusive disease of the liver after marrow transplantation. The clinical syndrome of hepatic venoocclusive disease vod after hematopoietic stem cell transplantation hsct is characterized by liver enlargement and pain, fluid retention, weight gain, and. It accounts for a significant fraction of transplantrelated mortality and, in its severe form, is almost always fatal. Understanding the similarities and differences between hepatic. 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. 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. Venoocclusive liver disease after bone marrow transplantation. Liver venoocclusive disease an overview sciencedirect. Immunohistochemical identification of the material within occluded central venules. When linked to hematopoietic transplant, over thirty percent cases prove to be fatal with a high mortality rate of the severe cases.
The pathophysiology of veno occlusive disease culminates in occlusion of the pulmonary blood vessels. Sinusoidal obstruction syndrome pathology outlines. Hepatic venoocclusive disease with immunodeficiency also called vodi is a hereditary disorder of the liver and immune system. Hepatic veno occlusive disease is a condition that blocks occludes small veins in the liver, disrupting blood flow in this organ. Hepatic venoocclusive disease an overview sciencedirect topics. The clinical syndrome of hepatic venoocclusive disease vod after haemopoietic stem cell transplantation sct is characterized by liver enlargement and pain, fluid. Lager veins and those affected by buddchiari syndrome are. Management of hepatic veno occlusive disease vod background. 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. Its signs and symptoms appear after the first few months of. This causes a decrease in blood flow inside the liver and.
Hepatic outflow obstruction, congestive hepatopathy, and the development of cirrhosis. Hepatic venoocclusive disease vod as a distinct clinical entity was first. 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. Sinusoidal obstruction syndrome sos, also known as venoocclusive disease vod. Pulmonary veno occlusive disease pvod is a rare form of pulmonary hypertension caused by progressive blockage of the small veins in the lungs. Hepatic venoocclusive diseaseimmunodeficiency syndrome is characterized by the association of severe hypogammaglobulinemia, combined t and b cell immunodeficiency, absent. The characteristic histological changes seen in vod have been studied extensively and are best understood in the setting of normal liver histology.
Hepatic venoocclusive disease induced by chinese medicinal herbs. Characterization of a reproducible rat model of hepatic venoocclusive disease laurie d. The pathology of noncirrhotic liver diseases causing portal hypertension such as extrahepatic portal obstruction, idiopathic portal hypertension, nodular regenerative hyperplasia, veno occlusive disease. Hepatic venoocclusive disease with immunodeficiency oxford. Wanless, in surgical pathology of the gi tract, liver, biliary tract, and pancreas second edition, 2009. Definition of hepatic venoocclusive disease nci dictionary.
Hepatic venoocclusive disease vod, also known as sinusoidal obstruction. Vod is defibrotide,84,85 a novel polydeoxyribonucleotide with adenosine. 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. The laboratory workup of a patient with possible venoocclusive disease has several objectives. Liver histology demonstrates obstruction of sinusoids in central areas with hepatocyte necrosis and hemorrhage.
Buddchiari syndrome bcs is a group of disorders caused by occlusion of the major hepatic veins or the inferior vena cava ivc or both at or near the level of the hepatic vein ostia. It is a complication of highdose chemotherapy given before a bone marrow transplant bmt and is marked by weight gain due to fluid r. Portal vein thrombosis and the histogenesis of veno centric cirrhosis, veno portal cirrhosis, and large regenerative nodules article feb 1998. Hepatic veno occlusive disease, or sinusoidal obstruction syndrome, is a common and potentially fatal complication following hsct. Hepatic veno occlusive disease hvod is a lifethreatening complication mostly occurring after highdose cytoreductive chemotherapy and hematopoietic stem cell transplantation hsct. Robbins and cotran pathologic basis of disease 2010, 8th ed. Vod can occur in as high as 70% of patients and is the most common hepatic complication in the immediate posttransplant period. Hepatic venoocclusive disease vod, increasingly referred to as sinusoidal obstruction syndrome, is a wellrecognized complication of hematopoietic stem cell transplantation and contributes to.
Hepatic venoocclusive disease hvod is a lifethreatening complication mostly occurring after highdose cytoreductive chemotherapy and hematopoietic stem cell transplantation hsct. Hepatic venoocclusive disease is a clinical syndrome characterized by hepatomegaly, ascites. Sinusoidal obstruction syndrome venoocclusive disease. 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. The clinical syndrome of hepatic veno occlusive disease vod after haemopoietic stem cell transplantation sct is characterized by liver enlargement and pain, fluid retention, weight gain, and jaundice mcdonald et al, 1984. Hepatic venoocclusive disease sinusoidal obstruction. 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.
Pulmonary venoocclusive disease in myeloproliferative. In most cases, affected individuals experience recurrent infections, but they may also suffer from autoimmune diseases and malignancies. Hepatic venoocclusive disease an overview sciencedirect. The immunodeficiency comprises severe hypogammaglobulinemia, clinical evidence of tcell immunodeficiency with normal numbers. The pathology of noncirrhotic liver diseases causing portal hypertension such as extrahepatic portal obstruction, idiopathic portal hypertension, nodular regenerative hyperplasia, veno occlusive disease, and buddchiari syndrome is mainly explained. Hepatic venoocclusive disease with immunodeficiency. Although a brief discussion of these disorders first appeared in a book by budd in 1845, lambron in 1842 is said to have reported the first case. Jul 01, 2006 hepatic veno occlusive 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 veno occlusive disease. 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 veno occlusive disease after bone marrow transplantation.
Venoocclusive hepatic disease differential diagnoses. This chapter focuses on hepatic veno occlusive disease with immunodeficiency vodi, including the historic and scientific background, clinical. 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. Liver venoocclusive disease an overview sciencedirect topics. 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. Hepatic veno occlusive disease is a lifethreatening condition in which toxic substances either chemotherapeutic drugs or pyrrolizidine alkaloids found in various herbal remedies cause severe damage to the endothelial cells of sinusoidal vessels in the liver, resulting in massive fibrosis and blood flow obstruction. Hepatic venoocclusive disease is caused by ingestion of certain species of plants belonging to the genera senecio, crotalaria, heliotropium, and cynoglossum. Toxic injury to liver sinusoids causes sloughing of endothelial cells which embolize to hepatic venules and cause eventual fibrosis of the venules. This causes a decrease in blood flow inside the liver and may lead to liver damage. The pathology, diagnosis, and treatment of hepatic veno. Revised diagnosis and severity criteria for sinusoidal. 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.
Along with graft versus host disease gvhd and cytomegalovirus cmv infection, venoocclusive disease vod or sinusoidal obstruction syndrome sos, is one of the most frequently encountered serious complications after stem cell transplantation. The lack of hepatic venoocclusive disease in other forms of inherited immunodeficiency suggest that this is a primary feature of vodi. What is hepatic venoocclusive diseasecausessymptoms. The clinical syndrome of hepatic venoocclusive disease vod after hematopoietic stem cell transplantation hsct is characterized by liver enlargement and pain, fluid retention, weight gain, and jaundice. Hepatic venoocclusive disease after hematopoietic stem cell. As a service to our customers we are providing this early version of the manuscript. Mar 31, 2016 the laboratory workup of a patient with possible veno occlusive disease has several objectives. Characterization of a reproducible rat model of hepatic. Severe vodsos, typically characterized by multiorgan failure, has been associated with a mortality rate greater than 80%. The pathology, diagnosis, and treatment of hepatic venoocclusive disease. Also called sinusoidal obstruction syndrome, vod is a group of disorders in which hepatic venous outflow obstruction is caused by subendothelial sclerosis of the sublobular hepatic veins, terminal hepatic venules, and sinusoids. The present study reports a case of biopsyproven pulmonary veno occlusive disease as a cause of severe pulmonary hypertension in a patient suffering from a chronic myeloproliferative disorder. Venoocclusive disease definition of venoocclusive disease. Hepatic venoocclusive disease, or sinusoidal obstruction syndrome, is a common and potentially fatal complication following hsct.
Clinical signs and symptoms of veno occlusive disease include hepatomegaly, right. The reported overall incidence rate of venoocclusive disease. Hepatic venoocclusive disease with immunodeficiency vodi is characterized by 1 primary immunodeficiency and 2 terminal hepatic lobular vascular occlusion and hepatic fibrosis. Hepatic venoocclusive disease vod, also known as sinusoidal obstruction syndrome sos, is a condition arising from occlusion of hepatic venules. Hepatic veno occlusive disease was examined by scanning electron microscopy sem. 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. Pathology of noncirrhotic liver disease springerlink. Hepatic veno occlusive disease vod or sinusoidal obstructive syndrome sos is a disorder characterised by obstruction of small intra hepatic venules and damage to the surrounding centrilobular hepatocytes and sinusoids. Primary immunodeficiency diseases are inherited disorders that affect human adaptive and innate immunity. Hepatic venoocclusive disease sinusoidal obstruction syndrome hepatic venoocclusive disease, which is also known as sinusoidal. Hepatic venoocclusive disease hepatic vod is a condition resulting from. Numerous novel tools show promise in facilitating the diagnosis of hvod. Veno occlusive disease in snow leopards panthera uncia. Lack of a reproducible animal model has hampered progress in understanding hepatic veno occlusive.
Pathology of noncirrhotic liver disease request pdf. Hepatic venoocclusive disease is a lifethreatening condition in which toxic substances either chemotherapeutic drugs or pyrrolizidine alkaloids found in various herbal remedies cause severe. Now it is considered an important cause of non cirrhotic portal. Nov 01, 2008 in severe cases, multiple organ failure or severe bacterial infections may occur. 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.
Sem correlated its histology and postmortem examination and disclosed microscopic occlusion of the centrilobular and sublobular veins in the liver, these veins were occluded partially or completely by intimal and medial thickening of their walls due to. Apr 09, 2018 the outlook for hepatic veno occlusive disease depends on the causative factor of the condition. The blockage leads to high blood pressures in the arteries of the lungs, which, in turn, leads to heart failure. Buddchiari syndrome and venoocclusive disease clinical gate. Hepatic venoocclusive disease radiology reference article. Veno occlusive disease sinusoidal obstruction syndrome veno occlusive disease is defined by the presence of prominent obstructive nonthrombotic lesions of the small hepatic. Hepatic venoocclusive disease originating in ecuador. Hepatic veno occlusive disease with immunodeficiency also called vodi is a hereditary disorder of the liver and immune system. The occurrence of hepatic veno occlusive disease vod has been reported in up to 60% of patients following stem cell transplantation sct, with incidence varying widely between studies depending on the type of transplant, conditioning regimen, and criteria used to make the diagnosis.
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. Although a brief discussion of these disorders first appeared in a book. 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. In most cases, affected individuals experience recurrent infections, but they may also suffer. Although its pathophysiology is not completely understood, the. The clinical syndrome of hepatic veno occlusive disease vod after hematopoietic stem cell transplantation sct is characterized by liver enlargement and pain, fluid retention, weight gain and jaundice 1, 2, 3. Pathology outlines sinusoidal obstruction syndrome. Hepatic venoocclusive disease genetic and rare diseases. In the western hemisphere, vod, classified as a conditioningrelated toxicity, is most commonly caused. Hepatic venoocclusive disease or sinusoidal obstruction syndrome.
Stone, in cardiovascular pathology fourth edition, 2016. The first goal, of course, is to confirm the diagnosis, the second goal is to look for a. Hepatic sinusoidal obstruction syndrome sos, previously termed hepatic venoocclusive disease vod, is one of the most feared complications of allogeneic and autologous hematopoietic cell. Rare, usually children and adolescents, especially females also after cancer chemotherapy causes pulmonary hypertension secondary to widespread thromboemboli of large and medium size.
Symptoms include hyperbilirubinemia, painful hepatomegaly and ascites. 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. This is a pdf file of an unedited manuscript that has been accepted for publication. In 2006, roscioli et al 1 described mutations in the. 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. Management of hepatic venoocclusive disease vod background. Sinusoidal obstruction syndrome hepatic veno occlusive. Nineteen patients had documented venoocclusive disease, nine had hepatic graftvshost disease gvhd five after proved venoocclusive disease, two had hepatitis, and 40 had no clinical or biochemical evidence of liver injury after bone marrow transplantation. Hepatic venoocclusive disease sinusoidal obstruction syndrome. Sinusoidal obstruction syndrome sos, also known as veno occlusive disease vod. Hepatic venoocclusive disease after hematopoietic stem. Mar 31, 2016 hepatic veno occlusive disease after bone marrow transplantation.
The outlook for hepatic venoocclusive disease depends on the causative factor of the condition. Previously called venoocclusive disease semin liver dis 2002. Hepatic veno occlusive disease vod, also known as sinusoidal obstruction syndrome sos, is a condition arising from occlusion of hepatic venules. 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. When linked to hematopoietic transplant, over thirty percent cases prove to be fatal with a. Hepatic venoocclusive disease with immunodeficiency genetic. Its signs and symptoms appear after the first few months of life. Hepatic venoocclusive disease or venoocclusive disease with immunodeficiency vodi is a condition in which some of the small veins in the liver are obstructed. Hepatic venoocclusive disease vod or sinusoidal obstructive syndrome sos is a disorder characterised by obstruction of small. Sinusoidal obstruction syndrome venoocclusive disease ncbi. Venoocclusive disease of the liver, also known as sinusoidal obstruction syndrome sos, is blockage of the very small microscopic veins in the liver. It is a complication of highdose chemotherapy given before a. Hepatic vod consists of occlusive fibrosis of small intrahepatc veins.
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