{"id":2131,"date":"2019-06-19T19:15:17","date_gmt":"2019-06-19T19:15:17","guid":{"rendered":"https:\/\/medicalcriteria.com\/web\/?p=1671"},"modified":"2026-02-03T19:19:15","modified_gmt":"2026-02-03T19:19:15","slug":"sos-vod","status":"publish","type":"post","link":"https:\/\/medicalcriteria.com\/web\/sos-vod\/","title":{"rendered":"Diagnosis and Severity Criteria for Sinusoidal Obstruction Syndrome\/Veno-Occlusive Disease in Children"},"content":{"rendered":"<div class=\"99c380e4b4a7b96c35d7ddf7dcb434e8\" data-index=\"1\" style=\"float: none; margin:0px 0 0px 0; text-align:center;\">\n<script async src=\"https:\/\/pagead2.googlesyndication.com\/pagead\/js\/adsbygoogle.js\"><\/script>\r\n<!-- MC 2019- Horizontal -->\r\n<ins class=\"adsbygoogle\"\r\n     style=\"display:block\"\r\n     data-ad-client=\"ca-pub-0127150553352455\"\r\n     data-ad-slot=\"3806776041\"\r\n     data-ad-format=\"auto\"\r\n     data-full-width-responsive=\"true\"><\/ins>\r\n<script>\r\n     (adsbygoogle = window.adsbygoogle || []).push({});\r\n<\/script>\n<\/div>\n<p>Hepatic sinusoidal obstruction syndrome\/veno-occlusive disease (SOS\/VOD) is a life-threatening complication of hematopoietic cell transplantation (HCT) that belongs to a group of diseases increasingly identified as transplant-related, systemic endothelial diseases. In most cases, SOS\/VOD resolves within weeks; however, severe SOS\/VOD results in multi-organ dysfunction\/failure with a mortality rate &gt;80%. <!--more--><\/p>\n<p><strong>EBMT diagnostic criteria for hepatic SOS\/VOD in children<\/strong><\/p>\n<ul>\n<li>No limitation for time of onset of SOS\/VOD<\/li>\n<\/ul>\n<p>The presence of two or more of the following<sup>a<\/sup><\/p>\n<ul>\n<li>Unexplained consumptive and transfusion-refractory thrombocytopenia<sup>b<\/sup><\/li>\n<li>Otherwise unexplained weight gain on three consecutive days despite the use of diuretics or a weight gain &gt;5% above baseline value<\/li>\n<li><sup>c<\/sup>Hepatomegaly (best if confirmed by imaging) above baseline value<\/li>\n<li><sup>c<\/sup>Ascites (best if confirmed by imaging) above baseline value<\/li>\n<li>Rising bilirubin from a baseline value on 3 consecutive days or bilirubin \u22652 mg\/dL within 72 h<\/li>\n<\/ul>\n<p>Abbreviations: CT= computed tomography; HCT= hematopoietic cell transplantation; MRI = magnetic resonance imaging; SOS\/VOD= sinusoidal obstruction<br \/>\nsyndrome\/veno-occlusive disease; US=ultrasonography; EBMT=European Society for Blood and Marrow Transplantation<\/p>\n<p><sup>a <\/sup>With the exclusion of other potential differential diagnoses.<br \/>\n<sup>b <\/sup>\u2265 1 weight-adjusted platelet substitution\/day to maintain institutional transfusion guidelines.<br \/>\n<sup>c<\/sup> Suggested: imaging (US, CT or MRI) immediately before HCT to determine baseline value for both hepatomegaly and ascites.<\/p>\n<p>&nbsp;<\/p>\n<p><strong>EBMT criteria for grading the severity of suspected hepatic SOS\/VOD in children<\/strong><\/p>\n<table style=\"border-collapse: collapse; width: 100%; height: 312px;\" border=\"1\">\n<tbody>\n<tr style=\"height: 24px;\">\n<td style=\"height: 48px; width: 152px;\" rowspan=\"2\">CTCAE<\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\">Mild<\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\">Moderate<\/td>\n<td style=\"height: 24px; width: 101px; text-align: center;\">Severe<\/td>\n<td style=\"height: 24px; width: 115px; text-align: center;\">Very severe MOD\/MOF<\/td>\n<\/tr>\n<tr style=\"height: 24px;\">\n<td style=\"height: 24px; width: 83px; text-align: center;\">1<\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\">2<\/td>\n<td style=\"height: 24px; width: 101px; text-align: center;\">3<\/td>\n<td style=\"height: 24px; width: 115px; text-align: center;\">4<\/td>\n<\/tr>\n<tr style=\"height: 24px;\">\n<td style=\"height: 24px; width: 152px;\">LFT<sup>b<\/sup> (ALT, AST, GLDH)<\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\">\u22642 \u00d7 normal<\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\">&gt;2 and \u22645 \u00d7 normal<\/td>\n<td style=\"height: 24px; width: 101px; text-align: center;\">&gt;5<\/td>\n<td style=\"height: 24px; width: 115px; text-align: center;\">&gt;5<\/td>\n<\/tr>\n<tr style=\"height: 24px;\">\n<td style=\"height: 24px; width: 152px;\">Persistent RT<sup>b<\/sup><\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\">&lt;3 days<\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\">3\u20137 days<\/td>\n<td style=\"height: 24px; width: 101px; text-align: center;\">&gt;7 days<\/td>\n<td style=\"height: 24px; width: 115px; text-align: center;\">&gt;7 days<\/td>\n<\/tr>\n<tr style=\"height: 24px;\">\n<td style=\"height: 24px; width: 152px;\">Bilirubin (mg\/dL)<sup>b<\/sup><\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\">&lt;2<\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\">&lt;2<\/td>\n<td style=\"height: 24px; width: 101px; text-align: center;\">\u22652<\/td>\n<td style=\"height: 24px; width: 115px; text-align: center;\">\u22652<\/td>\n<\/tr>\n<tr style=\"height: 24px;\">\n<td style=\"height: 24px; width: 152px;\">Bilirubin (\u03bcmol\/L)<\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\">&lt;34<\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\">&lt;34<\/td>\n<td style=\"height: 24px; width: 101px; text-align: center;\">\u226534<\/td>\n<td style=\"height: 24px; width: 115px; text-align: center;\">\u226534<\/td>\n<\/tr>\n<tr style=\"height: 24px;\">\n<td style=\"height: 24px; width: 152px;\">Ascites<sup>b<\/sup><\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\">Minimal<\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\">Moderate<\/td>\n<td style=\"height: 24px; width: 226px; text-align: center;\" colspan=\"2\">Necessity for paracentesis (external drainage)<\/td>\n<\/tr>\n<tr style=\"height: 24px;\">\n<td style=\"height: 24px; width: 152px;\">Bilirubin kinetics<\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\"><\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\"><\/td>\n<td style=\"height: 24px; width: 101px; text-align: center;\"><\/td>\n<td style=\"height: 24px; width: 115px; text-align: center;\">Doubling within 48 h<\/td>\n<\/tr>\n<tr style=\"height: 24px;\">\n<td style=\"height: 24px; width: 152px;\">Coagulation<\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\">Normal<\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\">Normal<\/td>\n<td style=\"height: 24px; width: 101px; text-align: center;\">Impaired coagulation<\/td>\n<td style=\"height: 24px; width: 115px; text-align: center;\">Impaired coagulation with need for replacement of coagulation factors<\/td>\n<\/tr>\n<tr style=\"height: 24px;\">\n<td style=\"height: 24px; width: 152px;\">Renal function GFR (mL\/min)<\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\">89\u201360<\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\">59\u201330<\/td>\n<td style=\"height: 24px; width: 101px; text-align: center;\">29\u201315<\/td>\n<td style=\"height: 24px; width: 115px; text-align: center;\">&lt;15 (renal failure)<\/td>\n<\/tr>\n<tr style=\"height: 48px;\">\n<td style=\"width: 152px; height: 48px;\">Pulmonary function (oxygen\u00a0requirement)<\/td>\n<td style=\"width: 83px; text-align: center; height: 48px;\">&lt;2 L\/min<\/td>\n<td style=\"width: 83px; text-align: center; height: 48px;\">&gt;2 L\/min<\/td>\n<td style=\"width: 226px; text-align: center; height: 48px;\" colspan=\"2\">Invasive pulmonary ventilation (including CPAP)<\/td>\n<\/tr>\n<tr style=\"height: 24px;\">\n<td style=\"height: 24px; width: 152px;\">CNS<\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\">Normal<\/td>\n<td style=\"height: 24px; width: 83px; text-align: center;\">Normal<\/td>\n<td style=\"height: 24px; width: 101px; text-align: center;\">Normal<\/td>\n<td style=\"height: 24px; width: 115px; text-align: center;\">New onset cognitive impairment<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<p>Abbreviations: ALT= alanine transaminase; AST= aspartate transaminase; CNS =central nervous system; CPAP=continuous positive airway pressure;<br \/>\nCTCAE =Common Terminology Criteria for Adverse Events; GFR =glomerular filtration rate; GLDH = glutamate dehydrogenase; LFT =liver function test; MOD\/<br \/>\nMOF= multi-organ dysfunction\/multi-organ failure; RT=refractory thrombocytopenia; SOS\/VOD, sinusoidal obstruction syndrome\/veno-occlusive disease.<\/p>\n<p>If patient fulfills criteria in different categories they must be classified in the most severe category. In addition, the kinetics of the evolution of cumulative symptoms within 48 h predicts severe disease.<\/p>\n<p><sup>b<\/sup> Presence of \u2265 2 of these criteria qualifies for an upgrade to CTCAE level 4 (very severe SOS\/VOD).<\/p>\n<p><sup>c<\/sup> Excluding pre-existent hyperbilirubinemia due to primary disease.<\/p>\n<p>&nbsp;<\/p>\n<p><strong>References:<\/strong><\/p>\n<ol>\n<li>Corbacioglu S, Carreras E, Ansari M, Balduzzi A, Cesaro S, Dalle JH, Dignan F, Gibson B, Guengoer T, Gruhn B, Lankester A, Locatelli F, Pagliuca A, Peters C, Richardson PG, Schulz AS, Sedlacek P, Stein J, Sykora KW, Toporski J, Trigoso E, Vetteranta K, Wachowiak J, Wallhult E, Wynn R, Yaniv I, Yesilipek A, Mohty M, Bader P. Diagnosis and severity criteria for sinusoidal obstruction syndrome\/veno-occlusive disease in pediatric patients: a new classification from the European society for blood and marrow transplantation.\u00a0Bone Marrow Transplant. 2018 Feb;53(2):138-145. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/28759025\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<li>Colecchia A, Ravaioli F, Sessa M, Alemanni VL, Dajti E, Marasco G, Vestito A, Zagari RM, Barbato F, Arpinati M, Cavo M, Festi D, Bonifazi F. Liver Stiffness Measurement Allows Early Diagnosis of Veno-Occlusive Disease\/Sinusoidal Obstruction Syndrome in Adult Patients Who Undergo Hematopoietic Stem Cell Transplantation: Results from a Monocentric Prospective Study.\u00a0Biol Blood Marrow Transplant. 2019 May;25(5):995-1003. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/30660772\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<li>Mohty M, Malard F, Alaskar AS, Aljurf M, Arat M, Bader P, Baron F, Bazarbachi A, Blaise D, Brissot E, Ciceri F, Corbacioglu S, Dalle JH, Dignan F, Huynh A, Kenyon M, Nagler A, Pagliuca A, Peri\u0107 Z, Richardson PG, Ruggeri A, Ruutu T, Yakoub-Agha I, Duarte RF, Carreras E. Diagnosis and severity criteria for sinusoidal obstruction syndrome\/veno-occlusive disease in adult patients: a refined classification from the European society for blood and marrow transplantation (EBMT). Bone Marrow Transplant. 2023 Jul;58(7):749-754. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/37095231\/\" target=\"_blank\" rel=\"noopener\">[Medline]<\/a><\/li>\n<\/ol>\n<p>&nbsp;<\/p>\n<p>Created Jun 12, 2019.<br \/>\nUp-date Feb 3, 2026.<\/p>\n\n<div style=\"font-size: 0px; height: 0px; line-height: 0px; margin: 0; padding: 0; clear: both;\"><\/div>","protected":false},"excerpt":{"rendered":"<p>Sorry, this entry is only available in Espa\u00f1ol.<\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_lmt_disableupdate":"no","_lmt_disable":"no","_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"footnotes":""},"categories":[171],"tags":[181,16,15,123,13,51,172,50,182,183,179,176,178,184,180,5,175,173,4,177,174],"class_list":["post-2131","post","type-post","status-publish","format-standard","hentry","category-hematology","tag-children","tag-criteria","tag-criterios","tag-diagnosis","tag-diagnostico","tag-disease","tag-ebmt","tag-enfermedad","tag-gravedad","tag-ninos","tag-obstruccion","tag-obstruction","tag-occlusive","tag-oclusiva","tag-severity","tag-sindrome","tag-sinusoidal","tag-sos","tag-syndrome","tag-veno","tag-vod"],"modified_by":"Guillermo Firman","_links":{"self":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/2131","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/comments?post=2131"}],"version-history":[{"count":4,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/2131\/revisions"}],"predecessor-version":[{"id":10617,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/2131\/revisions\/10617"}],"wp:attachment":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/media?parent=2131"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/categories?post=2131"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/tags?post=2131"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}