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 enzyme. Sos, also known as venoocclusive disease vod, occurs as a result of cytoreductive therapy prior to hematopoietic stem cell transplantation hsct, following oxaliplatin. Sinusoidal obstruction syndrome sos is a rare vascular disease of the liver, which can lead to lethal liver failure and portal hypertensionrelated complications. Revised diagnosis and severity criteria for sinusoidal obstruction syndromeveno occlusive disease in adult patients. Sinusoidal obstruction syndrome sos, previously known as venoocclusive disease vod. Case report fatal venoocclusive disease vodsinusoidal. Typical clinical presentations of sosvod include body weight gain, painful hepatomegaly, ascites, and jaundice 2,3. Yakushijin k, atsuta y, doki n, yokota a, kanamori h, miyamoto t, et al. Case report dactinomycininduced hepatic sinusoidal. This is the first reported case of oxaliplatininduced sinusoidal obstruction syndrome sos among the chinese. Hepatic venoocclusive disease sinusoidal obstruction. Sinusoidal obstruction syndrome posttreatment with.
Sinusoidal dilatation may actually be a nonspecific feature of impaired portal venous blood inflow, whatever the cause, or a feature of severe systemic inflammatory reaction syndrome, whatever the cause. Newer proposed name of hepatic sinusoidal obstruction syndrome schulman. Sinusoidal obstruction syndrome sos, also known as hepatic venoocclusive disease vod, is 1 of the lifethreatening complications after hematopoietic stem cell transplantation hsct 1. Fatal venoocclusive disease vodsinusoidal obstruction syndrome sos developing after highdose chemotherapy combined with autologous peripheral blood stem cell transplantation for recurrent malignant lymphoma. Sinusoidal obstruction syndrome definition of sinusoidal. Although it has been described after both autologous and allogeneic hsct, it seems much more frequent after the latter, with a reported incidence of 8% to 14% 1. Hepatic sinusoidal obstruction syndrome sos, previously termed. Rt decreased albumin production ascites 4 modified seattle criteria 1984 at least 2 of the following by day 20 baltimore criteria 1987 bilirubin.
The significance of nonobstructive sinusoidal dilatation. Sinusoidal obstruction syndrome sos is a lethal complication after hematopoietic stem cell transplantation hsct. Sinusoidal obstruction syndrome sos, also known as hepatic venoocclusive disease vod, is a frequent and serious toxic complication of hematopoietic stem cell transplantation hsct. Recent developments with defibrotide for the treatment of. Genetic predisposition resulting in sinusoidal obstruction. Treatment and prevention of hepatic sinusoidal obstruction. Diagnosis, grading, and treatment recommendations for. Pdf diagnosis and severity criteria for sinusoidal obstruction. Hepatic sinusoidal obstruction syndromevenoocclusive disease sosvod is a lifethreatening complication of hct that belongs to a group of diseases. Tusanqirelated sinusoidal obstruction syndrome in china.
Hepatic sinusoidal obstruction syndrome sos, previously termed hepatic venoocclusive disease vod, is one of the most feared complications of allogeneic and autologous hematopoietic cell transplantation hct. Unfortunately, such criteria are not highly specificity and fail to provide a timely. Diagnosis of hepatic sinusoidal obstruction syndrome veno. Defibrotide sodium for the treatment of hepatic veno. Its rapid and accurate diagnosis is crucial for patient survival. Sinusoidal obstruction syndrome sos, previously called venoocclusive disease vod, can be a devastating complication after hematopoietic cell transplantation hct. Hepatic venoocclusive diseasesinusoidal obstruction syndrome vodsos is an unpredictable condition associated with endothelialcell damage due to conditioning for hematopoietic stemcell transplantation hsct or chemotherapy without hsct. Sinusoidal obstruction syndrome sos is a kind of rare liver disease. A disruption in the normal flow of venous blood from the sinusoids of the liver and the hepatic venules. Incidence of anicteric venoocclusive diseasesinusoidal. Sinusoidal obstruction syndromevenoocclusive disease. Objectives determine the effectiveness and cost of defibrotide in patients with severe hepatic sinusoidal obstruction syndrome following haematopoietic stem cell transplantation in a tertiary hospital. Sinusoidal obstruction syndrome sos is a rare liver disorder due to hepatic vascular injury.
This typically results in a distinctive triad of clinical signs including hepatomegaly with right upper quadrant tenderness, third space fluid retention e. Defibrotide df is the only drug internationally recommended for sos treatment. Ld in north america and western europe, sos, or hepatic venoocclusive. Venoocclusvie disease vod sinusoidal obstruction syndrome sos occurs following injury to the hepatic venous endothelium, resulting in hepatic venous outflow obstruction due to occlusion of the hepatic venules and sinusoids. Vod, also known as sinusoidal obstruction syndrome sos, with renal or pulmonary dysfunction following hematopoietic stemcell transplantation hsct. Sinusoidal obstruction syndrome sos, previously known as veno occlusive disease vod, is a distinctive and potentially fatal form. Defibrotide for the treatment of severe hepatic sinusoidal.
Novel ultrasonographic scoring system of sinusoidal. Clinical characteristics, ct signs, and pathological. Dactinomycin, hepatic sinusoidal obstructive syndrome. Nacetylcysteine, nephroblastoma, vincristine introduction hepatic sinusoidal obstruction syndrome sos, previously known as venoocclusive disease, is a frequent complication with significant mortality after. Subsequent genetic testing revealed that this patient had the gstt1null and gstm1null genotype, known to be an independent risk factor for developing oxaliplatininduced sos. The chart showing pdf series, word series, html series, scan qr. Pdf sinusoidal obstruction syndromevenoocclusive disease. Sinusoidal obstruction syndrome, cd34, sinusoidal capillarization 1. Although refractory ascites is a relatively rare postoltcomplication,persistenceofalargeamountof.
Hepatic sinusoidal obstruction syndrome without preceding. Mortality in patients with vodsos and multiorgan dysfunction mod may be 80%. Revised diagnosis and severity criteria for sinusoidal. Hepatic sinusoidal obstruction syndrome hsos is a rare vascular disease of the liver. Noninvasive imaging diagnosis of sinusoidal obstruction. Differences in the incidence of genetic predisposition and clinical presentation of sinusoidal obstructive syndrome between children and adults have rendered the historical baltimore and. Patient 2 presented with intrahepatic portalhepatic venous shunt at 2 years and 6 months and portalsystemic shunt encephalopathy at 4 years and 11 months after tdm1 treatment. Adults or children treated with defibrotide at a mean dose of 6.
Hepatic sinusoidal obstruction syndrome sos, previously termed hepatic veno occlusive disease vod, is characterized by hepatomegaly. Rt inability of liver to clear broken down red cells ascites. Introduction hepatic sinusoidal obstruction syndrome sos, previously known as venoocclusive disease vod, is a distinctive and potentially fatal form of hepatic injury that occurs typically after exposure to drug or toxic stimuli. To get a better understanding of pasinduced hsos, a large sample of the patients 116 cases with pasinduced hsos were enrolled and we analyzed clinical profiles of pasrelated sos in china. The condition is also known as sinusoidal obstruction syndrome sos in view of the associated characteristic. Hepatic sinusoidal obstruction syndrome in a child after. It accounts for a significant fraction of transplantrelated mortality and, in its severe form, is almost always fatal. 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. Hepatic venoocclusive disease sinusoidal obstruction syndrome. Hsos following standard chemotherapy has been reported in patients.
Sinusoidal obstructive syndrome, also known as hepatic venoocclusive disease, is a potentially lifethreatening complication that occurs in children undergoing haemopoietic stemcell transplantation hsct. Sinusoidal obstruction syndrome, previously known as venoocclusive disease, is a distinctive and potentially fatal form of hepatic injury that occurs predominantly, if not only, after drug or toxin exposure. Hepatic venoocclusive 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, increasingly re ferred to as sinusoidal obstruction syndrome, is a well. It has a reported overall mean incidence of 8% to 14%, with an incidence of up to 60% in some highrisk populations 1, 2, 3. 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. Sinusoidal obstruction syndrome sos, previously known as venoocclusive disease, is characterized by concentric and nonthrombotic obstruction of the sinusoid and central vein lumen with no identified primitive or thrombotic hepatic vein lesions. Hepatic sinusoidal obstruction after gemtuzumab ozogamicin mylotarg therapy. Sos is often established clinically, based on baltimore, modified seattle, or european society for blood and marrow transplantation ebmt criteria. Introduction venoocclusive disease of the liver vod occurs as a result of the conditioning treatment administered prior to haemopoietic stem cell transplantation hsct. Richardson adanafarber cancer institute, harvard medical school, boston, ma, usa. Request pdf sinusoidal obstruction syndrome hepatic venoocclusive disease hepatic sinusoidal obstruction syndrome sos is an obliterative venulitis of the terminal hepatic venules, which. Manifestations and management of venoocclusive disease. Hepatic sinusoidal obstruction syndrome hsos, the new name given to venoocclusive disease vod of the liver, is a wellknown complication of highdose chemotherapy employed with hematopoietic stem cell transplantation, but it has rarely been observed in children who receive conventional chemotherapy.
Sinusoidal obstruction syndrome hepatic venoocclusive. Seminar sinusoidal obstruction syndrome hepatic veno. Sinusoidal obstruction syndrome venoocclusive disease. Hepatic sinusoidal obstruction syndrome caused by herbal. The change in the nomenclature of this disease is primarily because the major lesion is located at the hepatic sinusoids. Lsecs may sometimes be the initial target of injury in a condition referred to as sinusoidal obstruction syndrome sos, formerly hepatic venoocclusive disease, vod, which is described as a change of the sinusoid that may lead to hepatocyte hypoxia, with liver dysfunction and. Rt decreased albumin production ascites 4 modified seattle criteria 1984 at least 2 of the following by. Hepatic venoocclusive diseasesinusoidal obstruction syndrome vodsos is a potentially lifethreatening complication of hematopoietic cell transplantation hct.
Sinusoidal obstruction syndrome and nodular regenerative hyperplasia are frequent oxaliplatinassociated liver lesions and partially prevented by bevacizumab in patients with hepatic colorectal metastasis. Sinusoidal obstruction syndrome venoocclusive disease ncbi. Sinusoidal obstruction syndrome after allogeneic hematopoietic stem cell. Pathology outlines sinusoidal obstruction syndrome. Hepatic venoocclusive disease or venoocclusive disease with immunodeficiency vodi is a condition in which some of the small veins in the liver are obstructed. Review recent developments with defibrotide for the treatment of hepatic venoocclusive diseasesinusoidal obstruction syndrome christine duncan a, justine kahnb, stephan a. Hepatic sinusoidal obstruction syndrome hsos, formerly called hepatic venoocclusive disease, is a rare yet lifehepatic sinusoidal obstruction syndrome caused by herbal medicine. Current situation and perspectives a position statement from the european society for blood and marrow transplantation ebmt. Sinusoidal dilatation found in the absence of an impaired sinusoidal blood outflow has been so far of unclear significance. Sometimes, hsos is caused by the intake of pacontaining herbs. In both the patients, liver biopsies revealed sinusoidal obstruction syndrome sos. There is no known difference in the frequency of gstm1null genotype between.
Clinical effects of recombinant thrombomodulin and. Pdf sinusoidal obstruction syndrome or venoocclusive disease sosvod is a potentially lifethreatening complication of hematopoietic. The incidence of sosvod after hsct varies from 5% to 60% 410, depending on types. Incidence of sinusoidal obstruction syndrome following mylotarg gemtuzumab ozogamicin. Defibrotide for sinusoidal obstruction syndromeveno. It occurs primarily after hematopoietic stem cell transplantation and is characterized by enlargement of the liver, right upper quadrant pain, jaundice, and massive fluid retention in the. Sinusoidal obstruction syndrome sos, also known as venoocclusive disease vod. Although the overall incidence and severity has fallen significantly, severe sos remains a formidable problem.
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