Case report fatal venoocclusive disease vodsinusoidal. Painful hepatomegaly, ascites and jaundice are typical symptoms of sos. Noninvasive imaging diagnosis of sinusoidal obstruction. Manifestations and management of venoocclusive disease. Sinusoidal obstruction syndrome sos is a lethal complication after hematopoietic stem cell transplantation hsct. 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.
Recent developments with defibrotide for the treatment of. Hepatic venoocclusive disease or venoocclusive disease with immunodeficiency vodi is a condition in which some of the small veins in the liver are obstructed. Rt decreased albumin production ascites 4 modified seattle criteria 1984 at least 2 of the following by day 20 baltimore criteria 1987 bilirubin. Sinusoidal obstruction syndrome sos, previously known as venoocclusive disease vod, is a rare lifethreatening condition seen most commonly after hematopoietic stem cell transplantation hsct. Chronic treatment with probucol effectively inhibits progression of pulmonary. Sinusoidal obstruction syndrome sos, formerly named venoocclusive disease, is a wellknown complication of hematopoietic stem cell transplantation and ingestion of. Pathology outlines sinusoidal obstruction syndrome.
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. Sos is characterized by painful hepatomegaly, jaundice, ascites, fluid. Animals were operated 4 days after sos induction with monocrotaline and received either vascular occlusion in the form of pringle maneuver pm or hepatectomy lr. Sos, also known as venoocclusive disease vod, occurs as a result of cytoreductive therapy prior to hematopoietic stem cell transplantation hsct, following oxaliplatin. Fibrin and cellular debris can also combine within the endothelial pores. Sinusoidal obstruction syndrome sos is a severe complication of hematopoietic stem cell transplantation hsct. Current situation and perspectives a position statement from the european society for blood and marrow transplantation ebmt. Activated perisinusoidal cells were only found around markedly dilated sinusoids. Rev esp enferm digcomprehensive cardiac evaluation that excluded rightsided heart failure. Hepatic sinusoidal obstruction syndrome sos, previously termed hepatic venoocclusive 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. Sinusoidal obstruction syndrome sos is characterized by damage to small hepatic. Mar 31, 2016 yamada n, urahashi t, ihara y, sanada y, wakiya t, okada n, et al. Case report successful treatment of severe sinusoidal.
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. A reported side effect is sinusoidal obstruction syndrome sos, formerly known as venoocclusive disease, which is characterized by a spectrum of pathologic changes, from sinusoidal dilation, peri sinusoidal haemorrhage, peliosis and nodular. Hepatic venoocclusive disease vod, also known as sinusoidal obstruction syndrome sos, is a nonhematologic toxicity associated with bmt. Case report association of idiopathic hepatic sinusoidal. A disruption in the normal flow of venous blood from the sinusoids of the liver and the hepatic venules. Pdf sinusoidal obstruction syndrome or venoocclusive disease.
Postliver transplantation sinusoidal obstruction syndrome with refractory ascites induced by mycophenolate mofetil. Sinusoidal obstruction syndrome definition of sinusoidal. Hsos following standard chemotherapy has been reported in patients. Its rapid and accurate diagnosis is crucial for patient survival.
Venoocclusive disease sinusoidal obstruction syndrome after haematopoietic stem cell transplantation. Sos is often established clinically, based on baltimore, modified seattle, or european society for blood and marrow transplantation ebmt criteria. Sinusoidal obstruction syndrome venoocclusive disease. Sinusoidal obstruction syndromevenoocclusive disease. Hepatic sos after conventional chemotherapy is uncommon and is mostly limited to pediatric patients receiving dactinomycinbased chemotherapy for wilms tumor 3, 4. Sinusoidal obstruction syndrome sos, previously called venoocclusive disease vod, can be a devastating complication after hematopoietic cell transplantation hct.
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. Sinusoidal obstruction syndrome sos, previously known as venoocclusive. Aug 23, 2009 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. Vod of the liver, more recently called sinusoidal obstruction syndrome, may mimic bcs clinically, because both conditions involve hepatic venous outflow obstruction. 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. At this point an angio computed tomography excluded venous and arterial flow obstruction again. A 64yearold caucasian woman underwent allogeneic peripheral blood stemcell transplantation from her human leukocyte antigenidentical sister against aggressive. Sos, also known as venoocclusive disease vod, occurs as a result of cytoreductive. Sinusoidal obstruction syndrome sos, also called venoocclusive disease vod of the liver, is one of the most relevant complications of hepatic sinusoidal endothelial origin that appears early after haematopoietic cell transplantation hct.
In addition, the inciting injury in the sinusoidal obstruction syndrome previously known as venoocclusive disease originates in the sinusoidal endothelial cells but eventually involves the hepatic veins resulting in a vascular outflow obstruction pattern. Defibrotide in the treatment of hepatic venoocclusive disease. Sinusoidal obstruction syndrome is characterized by tender hepatomegaly, ascites, and weight gain. 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. 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. 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. Hepatic venoocclusive disease radiology reference article. Hepatic venoocclusive disease sinusoidal obstruction syndrome vodsos is a potentially lifethreatening complication of conditioning during hematopoietic stem cell transplantation hsct or. Oxaliplatin toxicity presenting as a liver nodule case.
Prophylactic, preemptive, and curative treatment for. Prophylactic, preemptive, and curative treatment for sinusoidal obstruction syndrome venoocclusive disease in adult patients. Unfortunately, such criteria are not highly specificity and fail to provide a timely. She improved completely, even after renal, cerebral and respiratory failure. Severe sinusoidal obstructive syndrome sos is a life. Sinusoidal obstruction syndrome hepatic venoocclusive. For successful prevention, identification, diagnosis and treatment of. Although the pathogenesis of oxaliplatin related sinusoidal obstructive syndrome is not fully understood, studies suggest it is. Damaged sinusoids can be associated with a partial or complete occlusion of small hepatic veins, hence the previous denomination of hepatic venoocclusive disease vod. Discerning sinusoidal obstruction syndrome venoocclusive. The incidence and severity of sinusoidal obstruction syndrome depends on the conditioning regimen used and presence of preexisting liver disease. Venoocclusive disease sinusoidal obstruction syndrome associated with potential antibodymediated rejection after pediatric living donor liver transplantation. Apr 10, 2015 oxaliplatin based chemotherapy is often used as adjuvant therapy in colon and rectal cancer or in perioperative therapy in liver metastatic disease 1,2.
Recently, an inherited condition combining vod and immunodeficiency. Revised diagnosis and severity criteria for sinusoidal obstruction syndromeveno occlusive disease in adult patients. Clinical effects of recombinant thrombomodulin and. A central pathogenic event is toxic destruction of hepatic sinusoidal central venous endothelial cells, and then the sloughed sinusoidal lining cells embolize downstream and obstruct sinusoidal flow 3, 4.
Clinical characteristics, ct signs, and pathological. 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. Reversible sinusoidal obstruction syndrome associated with. It is most commonly a complication of hematopoietic cell transplantation hct. Idiopathic pure hsd is frequently associated with the immunological features of the antiphospholipid syndrome. Mar 23, 2012 venoocclusive disease vod also called sinusoidal obstruction syndrome sos typically occurs within 21 days of an allogenic, hematopoietic stem cell transplant. Although the overall incidence and severity has fallen significantly, severe sos remains a formidable problem. Incidence of anicteric venoocclusive diseasesinusoidal. Al jefri ah, abujazar h, alahmari a, al rawas a, al zahrani z, alhejazi a, bekadja ma, ibrahim a, lahoucine m, ousia s, bazarbachi a. Sinusoidal dilatation was marked in six of 11 patients 54%, including two patients with antiphospholipid antibodies. Sinusoidal obstruction syndrome sos, also referred to as venoocclusive disease, is a rare liver vascular injury that is highly lethal. These patients may have markedly elevated lft, and frequently develop ascites because of increased sinusoidal pressures resulting from outflow obstruction. Mar 10, 2009 we report a case of sinusoidal obstruction syndrome, a typical and lifethreatening complication after allogeneic stemcell transplantation, successfully treated with defibrotide despite massive multiple organ failure. Hepatic venoocclusive disease sinusoidal obstruction syndrome vodsos is a potentially lifethreatening complication of hematopoietic cell transplantation hct.
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 is a rare liver vascular injury disease, characterized by damage to small hepatic vessels, affecting particularly the sinusoidal endothelium, which result in. Buddchiari syndrome and venoocclusive disease clinical gate. Sinusoidal obstruction syndrome sos, previously known as venoocclusive disease vod. When we look at the behavior of this ferris wheel it is clear that it completes 1 cycle, or 1 revolution, and then repeats this revolution over and over again. Sinusoidal obstruction syndrome hepatic venoocclusive disease. As a result, defibrotide has been recommended for treatment of sos vod. Periodic acidschiff and immunohistochemistry staining of normal liver and hsos hepatomegaly specimens with antibodies to egfr, pegfr tyr1068, nqo1, p62 and paktser 473. Sinusoidal obstruction syndrome in the animal model. Sinusoidal obstruction syndrome sos, formerly named venoocclusive disease, is a wellknown complication of hematopoietic stem cell transplantation and ingestion of food or drinks contaminated by. Hepatic venoocclusive disease vod, also known as sinusoidal obstruction syndrome sos, represents the most frequent complication in patients in early phase following hematopoietic stemcell.
Vod has an unpredictable course that makes early diagnosis and treatment crucial. Pyrrolizidine alkaloidsinduced hepatic sinusoidal obstruction. Strategies for the management of hepatic venoocclusive. 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 tewari is an assistant professor with the section of hematologyoncology at baylor college of medicine and texas childrens hospital in houston, texas. Vod, also known as sinusoidal obstruction syndrome sos, with renal or pulmonary dysfunction following hematopoietic stemcell transplantation hsct.
Vod also known as sinusoidal obstruction syndrome 5. Pdf sinusoidal obstruction syndromevenoocclusive disease. Also known as sinusoidal obstruction syndrome, the disease results from. Introduction we report a case of sinusoidal obstruction syndrome sos, a typical and lifethreatening complication after. Oct 30, 2014 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. This causes a decrease in blood flow inside the liver and may lead to liver damage. We report the case of a young man transplanted for sos. Nrf2 activates growth factor genes and downstream akt. Review recent developments with defibrotide for the treatment of hepatic venoocclusive disease sinusoidal obstruction syndrome christine duncan a, justine kahnb, stephan a. This retrospective study was conducted in japan to determine the incidence, risk factors and outcomes of sinusoidal obstruction syndrome sos after allogeneic hematopoietic stem cell. Sinusoidal obstruction syndrome sos in multiple myeloma.
Hepatic venoocclusive 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. Vod involves the sinusoids and the central and sublobular hepatic veins within the liver, however, rather than the hepatic veins kumar et al, 2003. Rt decreased albumin production ascites 4 modified seattle criteria 1984 at least 2 of the following by. Hepatic sinusoidal obstruction syndrome sos is an obliterative venulitis of the. Hepatic sinusoidal obstruction syndrome in a child after. Diagnosis and treatment of sinusoidal obstruction syndrome. Sinusoidal obstruction syndrome europe pmc article europe. Richardson adanafarber cancer institute, harvard medical school, boston, ma, usa. Sinusoidal obstruction syndrome sos is characterized by damage to small hepatic vessels affecting particularly sinusoidal endothelium. Hepatic sinusoidal obstruction syndrome hsos, also known as venoocclusive disease vod, is characterized by damage to sinusoidal endothelium 1, 2.
Wynn,2 nedim hadzic,3 john karani,4 alberto quaglia,5 antonio pagliuca,6 paul veys,7 and michael n. Revised diagnosis and severity criteria for sinusoidal obstruction syndrome venoocclusive disease in adult patients. Successful treatment of severe sinusoidal obstruction. Neoadjuvant chemotherapy, however, is associated with considerable sideeffects. Hepatic venoocclusive disease vod, also known as sinusoidal obstruction syndrome sos, is a condition arising from occlusion of hepatic venules. Postliver transplantation sinusoidal obstruction syndrome. Middle eastnorth africa regional consensus on prevention, diagnosis and management.
Fatal venoocclusive disease vod sinusoidal obstruction syndrome sos developing after highdose chemotherapy combined with autologous peripheral blood stem cell transplantation for recurrent malignant lymphoma. Sinusoidal obstruction syndrome sos, previously known as hepatic venoocclusive disease, is a rare disorder with the unique etiopathogenesis of toxic injury to hepatic sinusoids, which induces progressive fibrotic obliteration of centrilobular veins. By continuing to browse this site you agree to us using cookies as described in about cookies. Potter8 on behalf of the haematooncology task force of the british committee for standards in.
Hepatic sinusoidal obstruction syndrome is a wellrecognized complication after highdose chemotherapy in the setting of hematopoietic stem cell transplantation 1,2. 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 development of. Sinusoidal obstruction syndrome sos is a rare liver disorder due to hepatic vascular injury. Rt inability of liver to clear broken down red cells ascites. It accounts for a significant fraction of transplantrelated mortality and, in its severe form, is almost always fatal. 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. It is pathologically characterized by the damage of hepatic sinusoidal endothelial cells, impeded sinusoidal blood flow, congestive sinusoidal dilatation, and perisinusoidal fibrosis. Liver sinusoidal endothelial cell progenitor cells promote liver regeneration in rats article pdf available in the journal of clinical investigation 1224. Pdf liver sinusoidal endothelial cell progenitor cells. It is potentially lifethreatening and contributes significantly to transplantrelated toxicity, morbidity, and mortality.
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