MCID: HPT046
MIFTS: 57

Hepatic Veno-Occlusive Disease

Categories: Cardiovascular diseases, Endocrine diseases, Gastrointestinal diseases, Liver diseases, Rare diseases

Aliases & Classifications for Hepatic Veno-Occlusive Disease

MalaCards integrated aliases for Hepatic Veno-Occlusive Disease:

Name: Hepatic Veno-Occlusive Disease 12 74 52 58 54 43 15 71 32
Sinusoidal Obstruction Syndrome 52 58
Veno-Occlusive Disease 12 54
Hepatic Venoocclusive Disease 74
Hepatic Vein Thrombosis 71
Hepatic Vein Occlusion 71
Venoocclusive Disease 71
Budd-Chiari Syndrome 71

Characteristics:

Orphanet epidemiological data:

58
hepatic veno-occlusive disease
Inheritance: Not applicable; Prevalence: 1-5/10000 (Europe); Age of onset: All ages; Age of death: any age;

Classifications:

Orphanet: 58  
Rare hepatic diseases


External Ids:

Disease Ontology 12 DOID:0080177
MeSH 43 D006504
ICD10 32 K76.5
MESH via Orphanet 44 D006504
ICD10 via Orphanet 33 K76.5
UMLS via Orphanet 72 C0019156
Orphanet 58 ORPHA890
UMLS 71 C0019154 C0019156 C0856761 more

Summaries for Hepatic Veno-Occlusive Disease

NIH Rare Diseases : 52 The following summary is from Orphanet , a European reference portal for information on rare diseases and orphan drugs. Orpha Number: 890 Definition Hepatic veno-occlusive disease (hepatic VOD) is a condition resulting from toxic injury to the hepatic sinusoidal capillaries that leads to obstruction of the small hepatic veins. Epidemiology Prevalence is unknown but hepatic VOD is a rare disease. Endemic zones have been identified in which the disease is associated with the consumption of herbal tea containing pyrrolizidine alkaloids. Epidemics affecting several hundred individuals may occur after contamination of the harvest with these plants. Clinical description It affects children and adults. The clinical picture is characterized by painful hepatomegaly, jaundice , and fluid retention that manifests by weight gain, edemas, and ascites. Hepatic insufficiency manifesting as coagulopathy and hepatic encephalopathy may occur. Functional renal insufficiency is common. In severe cases, multiple organ failure or severe bacterial infections may occur.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 VOD may also occur after chemotherapy or radiation therapy . Etiology Hepatic sinusoidal endothelial cell lesions appear to be the primary cause of the disease, leading to non-thrombotic occlusion of hepatic veins with concentric subendothelial thickening associated with edema and eventually fibrosis. Diagnostic methods Diagnosis is based on recognition of the clinical manifestations (hepatomegaly, jaundice, and weight gain) and their association with a potential cause of hepatic VOD (hematopoietic stem cell transplantation or chemotherapy, exposure to pyrrolizidine alkaloids), together with exclusion of other causes of liver disease. Liver biopsy is often indicated. Imaging studies, particularly hepatic Doppler-ultrasonography , may help to confirm the diagnosis. Differential diagnosis Differential diagnoses include other causes of acute or chronic liver disease, and, in the case of hematopoietic stem cell transplantation, graft-versus-host disease and severe infections. Management and treatment No specific treatment is available. Defibrotide is frequently used, mainly on the basis of limited studies (historic comparisons) and because side effects are uncommon. Preventive treatment with heparin is often indicated, but its efficacy has not been proven. In case of hematopoietic stem cell transplantation, the main prophylactic treatment is the use of less hepatotoxic conditioning regimens. Prognosis Severity and course of the disease vary between patients. In severe cases, the prognosis is poor with a high mortality rate (up to 90%) due to multiple organ failure. Visit the Orphanet disease page for more resources.

MalaCards based summary : Hepatic Veno-Occlusive Disease, also known as sinusoidal obstruction syndrome, is related to pulmonary edema and portal vein thrombosis. An important gene associated with Hepatic Veno-Occlusive Disease is SERPINC1 (Serpin Family C Member 1), and among its related pathways/superpathways are Response to elevated platelet cytosolic Ca2+ and Formation of Fibrin Clot (Clotting Cascade). The drugs Defibrotide and Busulfan have been mentioned in the context of this disorder. Affiliated tissues include liver, bone and bone marrow, and related phenotypes are hepatomegaly and renal insufficiency

Disease Ontology : 12 A hepatic vascular disease that is characterized by obstruction of some of the small veins of the liver.

Wikipedia : 74 Hepatic veno-occlusive disease or veno-occlusive disease with immunodeficiency (VODI) is a condition in... more...

Related Diseases for Hepatic Veno-Occlusive Disease

Diseases related to Hepatic Veno-Occlusive Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 459)
# Related Disease Score Top Affiliating Genes
1 pulmonary edema 31.1 SERPINC1 CXCL8 CSF3
2 portal vein thrombosis 31.0 SERPINE1 SERPINC1 PLAT
3 portal hypertension 30.9 VEGFA THPO SERPINE1 F3 CXCL8
4 idiopathic interstitial pneumonia 30.7 VEGFA SERPINE1 CXCL8
5 protein c deficiency 30.6 THBD SERPINE1 SERPINC1
6 ischemic colitis 30.6 VEGFA SERPINE1 SERPINC1
7 thrombotic thrombocytopenic purpura 30.5 THBD SERPINC1 PLAT F3
8 cardiac tamponade 30.5 PLAT F8 F3
9 toxic shock syndrome 30.5 SERPINC1 F3 CXCL8
10 hemolytic-uremic syndrome 30.5 THBD F3 CXCL8
11 purpura 30.5 THPO THBD SERPINC1 F3
12 endocarditis 30.4 SERPINE1 SERPINC1 PLAT CXCL8
13 mucositis 30.4 VEGFA CSF3 ABCB1
14 adult respiratory distress syndrome 30.3 THBD SERPINE1 CXCL8 CSF3
15 thrombophilia due to thrombin defect 30.3 THBD SERPINE1 SERPINC1 PLAT F8 F3
16 pulmonary embolism 30.2 THBD SERPINE1 SERPINC1 PLAT F8 F3
17 nodular regenerative hyperplasia 30.2 TPMT F3
18 engraftment syndrome 30.2 CXCL8 CSF3 ABCB1
19 castleman disease 30.2 VEGFA CXCL8 CSF3
20 liver cirrhosis 30.1 THPO SLC17A5 SERPINC1 HFE F3
21 exanthem 30.1 VEGFA F3 CXCL8 CSF3
22 disseminated intravascular coagulation 30.0 THBD SERPINE1 SERPINC1 PLG PLAT F3
23 thrombosis 30.0 THBD SERPINE1 SERPINC1 PLG PLAT F8
24 thrombophlebitis 29.9 SERPINE1 SERPINC1 PLG PLAT F8 F3
25 liver disease 29.7 THPO SLC17A5 HFE F3 CXCL8
26 antiphospholipid syndrome 29.6 THBD SULT1A3 SERPINE1 SERPINC1 PLAT F3
27 varicose veins 29.6 VEGFA THBD SERPINC1 PLAT HFE FGF2
28 childhood acute lymphocytic leukemia 29.4 VEGFA TPMT FGF2 ABCB1
29 acute leukemia 29.3 THPO CSF3 CD33 ABCB1
30 pancytopenia 29.2 TPMT THPO CSF3 CD33
31 pulmonary hypertension 29.1 VEGFA THBD SERPINE1 SERPINC1 PLG PLAT
32 vascular disease 29.0 VEGFA THBD SERPINE1 SERPINC1 PLG PLAT
33 inflammatory bowel disease 29.0 VEGFA TPMT SERPINC1 FGF2 F3 CXCL8
34 leukemia, chronic myeloid 28.9 VEGFA THPO CSF3 CD33 ABCB1
35 leukemia, acute myeloid 28.9 VEGFA THPO FGF2 CXCL8 CSF3 CD33
36 hematologic cancer 28.9 VEGFA THPO CSF3 CD33 ABCB1
37 myeloma, multiple 28.9 VEGFA FGF2 CXCL8 CSF3 CD33 ABCB1
38 systemic lupus erythematosus 28.8 VEGFA THBD SULT1A3 SERPINE1 F3 CXCL8
39 thrombocytopenia 28.8 THPO THBD SERPINE1 SERPINC1 PLAT F3
40 aplastic anemia 28.6 VEGFA TPMT THPO THBD SERPINE1 SERPINC1
41 leukemia, acute lymphoblastic 28.5 TPMT THPO SERPINC1 HFE CSF3 CD33
42 hepatic vascular disease 28.5 VEGFA THPO SERPINE1 SERPINC1 PLG F3
43 myelodysplastic syndrome 28.3 VEGFA THPO HFE FGF2 CXCL8 CSF3
44 hepatic venoocclusive disease with immunodeficiency 12.9
45 pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis 12.8
46 pulmonary hypertension, primary, 1 10.9
47 graft-versus-host disease 10.7
48 bilirubin metabolic disorder 10.6
49 cardiac tuberculosis 10.6 SERPINE1 SERPINC1
50 mercury poisoning 10.6 THBD PLAT

Comorbidity relations with Hepatic Veno-Occlusive Disease via Phenotypic Disease Network (PDN):


Heart Disease

Graphical network of the top 20 diseases related to Hepatic Veno-Occlusive Disease:



Diseases related to Hepatic Veno-Occlusive Disease

Symptoms & Phenotypes for Hepatic Veno-Occlusive Disease

Human phenotypes related to Hepatic Veno-Occlusive Disease:

58 31 (show all 12)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hepatomegaly 58 31 hallmark (90%) Very frequent (99-80%) HP:0002240
2 renal insufficiency 58 31 hallmark (90%) Very frequent (99-80%) HP:0000083
3 ascites 58 31 hallmark (90%) Very frequent (99-80%) HP:0001541
4 abdominal pain 58 31 hallmark (90%) Very frequent (99-80%) HP:0002027
5 elevated hepatic transaminase 58 31 hallmark (90%) Very frequent (99-80%) HP:0002910
6 jaundice 58 31 hallmark (90%) Very frequent (99-80%) HP:0000952
7 respiratory failure 58 31 hallmark (90%) Very frequent (99-80%) HP:0002878
8 increased body weight 58 31 hallmark (90%) Very frequent (99-80%) HP:0004324
9 increased total bilirubin 58 31 hallmark (90%) Very frequent (99-80%) HP:0003573
10 prolonged partial thromboplastin time 58 31 frequent (33%) Frequent (79-30%) HP:0003645
11 hepatic encephalopathy 58 31 very rare (1%) Very rare (<4-1%) HP:0002480
12 abnormality of coagulation 58 Frequent (79-30%)

MGI Mouse Phenotypes related to Hepatic Veno-Occlusive Disease:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 10.21 ABCB1 CSF3 F3 F8 FGF2 HFE
2 homeostasis/metabolism MP:0005376 10.17 ABCB1 F3 F8 FGF2 HFE PLAT
3 immune system MP:0005387 10.03 ABCB1 CSF3 F3 F8 HFE PLAT
4 mortality/aging MP:0010768 9.77 ABCB1 F3 F8 FGF2 HFE PLAT
5 liver/biliary system MP:0005370 9.7 ABCB1 HFE PLG SERPINC1 SERPINE1 THBD
6 reproductive system MP:0005389 9.28 ABCB1 F8 FGF2 PLAT PLG SERPINC1

Drugs & Therapeutics for Hepatic Veno-Occlusive Disease

Drugs for Hepatic Veno-Occlusive Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 179)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Defibrotide Approved, Investigational Phase 4 83712-60-1
2
Busulfan Approved, Investigational Phase 4 55-98-1 2478
3
Fludarabine Approved Phase 4 21679-14-1, 75607-67-9 30751
4
Treosulfan Investigational Phase 4 299-75-2 9296
5 Fibrinolytic Agents Phase 4
6 Platelet Aggregation Inhibitors Phase 4
7 Alkylating Agents Phase 4
8 Antineoplastic Agents, Immunological Phase 4
9 Immunoglobulins Phase 4
10 Antibodies Phase 4
11 Gemtuzumab Phase 4
12
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3 83-43-2 6741
13
Methylprednisolone hemisuccinate Approved Phase 2, Phase 3 2921-57-5
14
leucovorin Approved Phase 3 58-05-9 6006 143
15
Prednisolone Approved, Vet_approved Phase 2, Phase 3 50-24-8 5755
16 Prednisolone acetate Approved, Vet_approved Phase 2, Phase 3 52-21-1
17
Hydrocortisone Approved, Vet_approved Phase 3 50-23-7 5754
18
Prednisolone phosphate Approved, Vet_approved Phase 2, Phase 3 302-25-0
19
Hydrocortisone acetate Approved, Vet_approved Phase 3 50-03-3
20
Cytarabine Approved, Experimental, Investigational Phase 3 147-94-4, 65-46-3 6253
21
Cyclophosphamide Approved, Investigational Phase 3 6055-19-2, 50-18-0 2907
22
Dalteparin Approved Phase 3 9005-49-6
23
Heparin Approved, Investigational Phase 3 9005-49-6 772 46507594
24
Daunorubicin Approved Phase 3 20830-81-3 30323
25
Pegaspargase Approved, Investigational Phase 3 130167-69-0
26
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
27
Vincristine Approved, Investigational Phase 3 57-22-7, 2068-78-2 5978
28
Doxorubicin Approved, Investigational Phase 3 23214-92-8 31703
29
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 3 1177-87-3
30
Mercaptopurine Approved Phase 3 50-44-2 667490
31
Dexamethasone Approved, Investigational, Vet_approved Phase 3 50-02-2 5743
32
Cobalt Approved, Experimental Phase 3 7440-48-4 104729
33
Thioguanine Approved Phase 3 154-42-7 2723601
34 Ethiodized oil Approved, Investigational Phase 3 8008-53-5
35
Iron Approved, Experimental Phase 3 7439-89-6, 15438-31-0 23925 27284
36
Alprostadil Approved, Investigational Phase 2, Phase 3 745-65-3 149351 5280723
37
Melphalan Approved Phase 3 148-82-3 4053 460612
38
alemtuzumab Approved, Investigational Phase 3 216503-57-0
39
Hydroxyurea Approved Phase 3 127-07-1 3657
40
Vidarabine Approved, Investigational Phase 3 24356-66-9 32326 21704
41
Norgestimate Approved, Investigational Phase 3 35189-28-7 6540478
42
Polyestradiol phosphate Approved Phase 3 28014-46-2
43
Ethinyl Estradiol Approved Phase 3 57-63-6 5991
44
Moxifloxacin Approved, Investigational Phase 3 354812-41-2, 151096-09-2 152946
45
Estradiol Approved, Investigational, Vet_approved Phase 3 50-28-2 5757
46
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
47
Prednisolone hemisuccinate Experimental Phase 2, Phase 3 2920-86-7
48 Anticoagulants Phase 3
49 Coagulants Phase 3
50 Anti-Inflammatory Agents Phase 3

Interventional clinical trials:

(show top 50) (show all 93)
# Name Status NCT ID Phase Drugs
1 Defibrotide in the Human Endotoxemia Model -- an Exploratory Trial Investigating the Effects and the Mechanisms of Defibrotide Completed NCT02876601 Phase 4 Defibrotide;Placebo (0.9% sodium chloride);Lipopolysaccharide;Placebo (0.9% sodium chloride bolus)
2 Using Ultrasound Elastography to Predict Development of Sinusoidal Obstruction Syndrome Recruiting NCT03858530 Phase 4
3 A PHASE 4, OPEN-LABEL, RANDOMIZED STUDY OF TWO INOTUZUMAB OZOGAMICIN DOSE LEVELS IN ADULT PATIENTS WITH RELAPSED OR REFRACTORY B-CELL ACUTE LYMPHOBLASTIC LEUKEMIA ELIGIBLE FOR HEMATOPOIETIC STEM CELL TRANSPLANTATION AND WHO HAVE RISK FACTOR(S) FOR VENO-OCCLUSIVE DISEASE Recruiting NCT03677596 Phase 4 inotuzumab ozogamicin-dose level 2;Inotuzumab ozogamicin-dose level 1
4 A SINGLE ARM, OPEN-LABEL, PHASE 4 STUDY EVALUATING QT INTERVAL, PHARMACOKINETICS, AND SAFETY OF GEMTUZUMAB OZOGAMICIN (MYLOTARG (TRADEMARKER)) AS A SINGLE-AGENT REGIMEN IN PATIENTS WITH RELAPSED OR REFRACTORY CD33-POSITIVE ACUTE MYELOID LEUKEMIA Recruiting NCT03727750 Phase 4 Gemtuzumab Ozogamicin
5 Allogeneic Stem Cell Transplant for Children and Adolescents With Acute Lymoblastic Leukemia FORUM - Pharmacogenomic Study (add-on Study) Recruiting NCT02670564 Phase 4
6 Validating Ultrasound Biomarkers for Hepatic Sinusoidal Obstruction Syndrome in Pediatric Hematopoietic Cell Transplant Patients Not yet recruiting NCT03963999 Phase 4 Contrast Enhanced Ultrasound (CEUS)
7 A Pilot/Feasibility Study of Ledipasvir/Sofosbuvir as Treatment for Hepatitis C in Hematopoietic Cell Transplantation (HCT) Recipients. Withdrawn NCT03279133 Phase 4 Ledipasvir 90mg/Sofosubvir 400mg
8 Prospective Randomized Study of the Incidence and Outcome of Veno-Occlusive Disease (VOD) With the Prophylactic Use of Defibrotide (DF) in Pediatric Stem Cell Transplantation Completed NCT00272948 Phase 2, Phase 3 Defibrotide;Defibrotide;Defibrotide
9 Defibrotide for Patients With Hepatic Veno-occlusive Disease: A Treatment IND Study Completed NCT00628498 Phase 3 Defibrotide;Defibrotide
10 Defibrotide for the Treatment of Severe Hepatic Veno-Occlusive Disease in Hematopoetic Stem Cell Transplant Patients: A Historically-Controlled, Multi-Center Phase 3 Study to Determine Safety & Efficacy Completed NCT00358501 Phase 3 Defibrotide
11 Phase III Double-Blind Trial Comparing Low-Molecular Weight Heparin (LMWH) Versus Placebo in Patients With Advanced Cancer Completed NCT00003674 Phase 3 dalteparin;standard therapy
12 Randomized Comparisons of Oral Mercaptopurine vs Oral Thioguanine and IT Methotrexate vs ITT for Standard Risk Acute Lymphoblastic Leukemia Completed NCT00002744 Phase 3 asparaginase;cyclophosphamide;cytarabine;daunorubicin hydrochloride;dexamethasone;doxorubicin hydrochloride;mercaptopurine;methotrexate;pegaspargase;prednisone;therapeutic hydrocortisone;thioguanine;vincristine sulfate
13 A Randomized and Multicenter Trial for Hepatocellular Carcinoma Adjuvant Treatment by Lipiocis Completed NCT00116454 Phase 3 131 I-lipiodol
14 A Phase III Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Effects of Fragmin (5,000 IU Subcutaneously) in Preventing Catheter-Related Complications When Given Daily to Cancer Patients With Central Venous Catheters Completed NCT00006083 Phase 3 Fragmin
15 The Unrelated Donor Marrow Transplantation Trial Completed NCT00003187 Phase 2, Phase 3 cyclophosphamide;cyclosporine;cytarabine;methotrexate;methylprednisolone
16 Randomized Study Comparing i.v. Busulfan (Busilvex®) Plus Fludarabine (BuFlu) Versus Busilvex® Plus Cyclophosphamide (BuCy2) as Conditioning Regimens Prior AlloHSCT in Patients (Age >= 40 and =<65 Years) With AML in Complete Remission. Completed NCT01191957 Phase 3 Busulphan plus Cyclophosphamide;Busulphan plus Fludarabine
17 A Phase 3, Randomized, Adaptive Study Comparing the Efficacy and Safety of Defibrotide vs Best Supportive Care in the Prevention of Hepatic Veno-Occlusive Disease in Adult and Pediatric Patients Undergoing Hematopoietic Stem Cell Transplant Recruiting NCT02851407 Phase 3 Defibrotide
18 Pharmacokinetic Study of Lipo-PGE1 for Prevention of VOD After HSCT Active, not recruiting NCT02338440 Phase 2, Phase 3 lipoprostaglandin E1
19 Reduced Intensity Stem Cell Transplantation for Sickle Cell Anemia in Patients 2-30 Years Old Active, not recruiting NCT01877837 Phase 3 Alemtuzumab;Fludarabine;Melphalan
20 Randomized, Double Blinded, Placebo-Controlled Trial of Antibacterial Prophylaxis for the Prevention of Bacterial Infections in the Post-Engraftment Phase After Allogeneic Hematopoeitic Stem Cell Transplantation Terminated NCT00324324 Phase 3 moxifloxacin hydrochloride;Placebo
21 Hematopoietic Stem Cell Transplantation With Targeted Busulfan and Fludarabine in Pediatric AML Unknown status NCT01274195 Phase 2 Busulfan
22 Gentuzumab Ozogamicin Berfore Allogeneic Stem Cell Transplantation in Patients With Relapsed CD33+ Acute Myeloid Leukemia Unknown status NCT00460447 Phase 1, Phase 2 Gemtuzumab Ozogamicin
23 HLA-Identical Sibling Donor Bone Marrow Transplantation for Individuals With Severe Sickle Cell Disease Using a Reduced Intensity Conditioning Regimen Unknown status NCT02776202 Phase 2 Fludarabine monophosphate
24 Chemotherapy Toxicity Reduction Via Urea Cycle Support Completed NCT00223730 Phase 2 Citrulline;Placebo
25 Phase II Trial of Pentostatin and Targeted Busulfan as a Novel Reduced Intensity Regimen for Allogeneic Hematopoietic Stem Cell Transplantation Using Laboratory-Guided (CD4-guided) Immunosuppression. Completed NCT00496340 Phase 2 Pentostatin;Busulfan;Rituximab
26 Defibrotide for Hematopoietic Stem Cell Transplant Patients With Severe Hepatic Venocclusive Disease: A Phase I/II Study to Determine the Minimal Effective Dose Completed NCT00003966 Phase 2 defibrotide
27 PERIOPERATIVE TREATMENT WITH COI-B (CAPECITABINE, OXALIPLATIN, IRINOTECAN AND BEVACIZUMAB) OF HIGH RISK OR BORDERLINE RESECTABLE COLORECTAL CANCER LIVER METASTASES Completed NCT02086656 Phase 2 capecitabine, oxaliplatin, irinotecan and bevacizumab
28 Adjunct Targeted Biologic Inhibition in Children With Multivessel Intraluminal Pulmonary Vein Stenosis Completed NCT00891527 Phase 1, Phase 2 Bevacizumab (Avastin) and Imatinib Mesylate (Gleevec)
29 Targeted Intensification by a New Preparative Regimen for Patients With Low-Grade B-Cell Lymphoma Utilizing Standard-Dose Yttrium-90 Ibritumomab Tiuxetan (Zevalin) Radioimmunotherapy (RIT) Combined With High-Dose Beam Followed by Autologous Stem Cell Transplantation (ASCT) Completed NCT00138086 Phase 2 Zevalin plus BEAM
30 A Multicenter Study of Unrelated Umbilical Cord Blood as an Alternate Source of Stem Cells for Transplantation Completed NCT00003913 Phase 2 busulfan;cyclophosphamide;methylprednisolone
31 Idarubicin and Ara-C in Combination With Gemtuzumab-Ozogamicin (IAGO) for Young Untreated Patients, Without an HLA Identical Sibling, With High Risk MDS or AML Developing After a Preceding Period With MDS During 6 Months Duration: A Phase II Study Completed NCT00077116 Phase 2 busulfan;cyclophosphamide;cytarabine;gemtuzumab ozogamicin;idarubicin
32 A Phase II Trial of Total Body Irradiation Plus Metabolism-Based Cyclophosphamide Dosing as Preparative Therapy for Allogeneic Hematopoietic Cell Transplant for Patients With Hematological Malignancy Completed NCT00317785 Phase 2 cyclophosphamide;cyclosporine;methotrexate;mycophenolate mofetil;sirolimus;tacrolimus
33 Unrelated Umbilical Cord Blood As An Alternate Source Of Stem Cells Transplantation Completed NCT00055653 Phase 2 busulfan;cyclophosphamide;cyclosporine;fludarabine phosphate;melphalan;methylprednisolone
34 A Phase I/II, Multi-Center, Open Label Study of Melphalan, Prednisone, Thalidomide and Defibrotide in Advanced and Refractory Multiple Myeloma Patients Completed NCT00406978 Phase 1, Phase 2 Prednisone;Melphalan;Thalidomide;Defibrotide
35 A Phase II Study of Sirolimus, Tacrolimus and Thymoglobulin®, as Graft-versus-Host- Disease Prophylaxis in Patients Undergoing Unrelated Donor Hematopoietic Cell Transplantation Completed NCT00691015 Phase 2 busulfan;carmustine;cyclophosphamide;cytarabine;etoposide;fludarabine phosphate;melphalan;anti-thymocyte globulin IV
36 Allogeneic Hematopoietic Cell Transplantation for Patients With Hematologic Disorders Who Are Ineligible or Inappropriate for Treatment With a More Intensive Therapeutic Regimen Completed NCT00448201 Phase 2 busulfan;fludarabine phosphate;methotrexate;tacrolimus
37 A Pilot Trial of Using Pre-Transplant Risk Stratification and Prophylactic Defibrotide to Prevent Serious Thrombotic Microangiopathy in High-Risk Hematopoietic Stem Cell Transplant Patients Recruiting NCT03384693 Phase 2 Defibrotide
38 Inotuzumab Ozogamicin Post-Transplant for Acute Lymphocytic Leukemia Recruiting NCT03104491 Phase 1, Phase 2 Inotuzumab Ozogamicin
39 Inotuzumab Ozogamicin for Children With MRD Positive CD22+ Lymphoblastic Leukemia Recruiting NCT03913559 Phase 2 Inotuzumab ozogamicin;Methotrexate;Hydrocortisone;Cytarabine;Diphenhydramine;Acetaminophen;Methylprednisolone
40 A Phase II Study of Inotuzumab Ozogamicin Followed by Blinatumomab for Ph-Negative CD22-Positive B-Lineage Acute Lymphoblastic Leukemia in Newly Diagnosed Older Adults or Adults With Relapsed or Refractory Disease Recruiting NCT03739814 Phase 2
41 Targeted Busulfan, Fludarabine Conditioning Regimen for Hematopoietic Stem Cell Transplantation in Genetic Rare Disease Recruiting NCT02034630 Phase 1, Phase 2 Busulfan
42 Targeted Busulfan, Fludarabine, Etoposide Conditioning Regimen for Hematopoietic Stem Cell Transplantation in Childhood and Adolescent Acute Lymphoblastic Leukemia Recruiting NCT02047578 Phase 2 Busulfan
43 A Dose Escalation Study of Intensity Modulated Total Marrow Irradiation (IMRT-TMI) With Fludarabine as a Myeloablative Conditioning Regimen for Allogeneic Hematopoietic Stem Cell Transplantation for Patients With Relapsed and Refractory Hematologic Malignancies Recruiting NCT03696537 Phase 1, Phase 2 Fludarabine
44 A Comparison of Reduced Dose Total Body Irradiation (TBI) and Cyclophosphamide With Fludarabine and Melphalan Reduced Intensity Conditioning in Adults With Acute Lymphoblastic Leukaemia (ALL) in Complete Remission. (ALL-RIC) Recruiting NCT03821610 Phase 2 Fludarabine;Melphalan;Alemtuzumab;Cyclophosphamide;Mesna
45 A Phase II Trial of Response-Adapted Second-Line Therapy for Hodgkin Lymphoma Using Anti-PD-1 Antibody Nivolumab ? ICE Chemotherapy as a Bridge to Autologous Hematopoietic Cell Transplant (NICE Trial) Recruiting NCT03016871 Phase 2 Carboplatin;Etoposide;Ifosfamide
46 Antithrombin-III for Patients With Hepatic Veno-occlusive Diseases Following Hematopoietic Stem Cell Transplantation Active, not recruiting NCT01886248 Phase 2 Freeze-dried Concentrated Human Antithrombin Ⅲ 500 IU
47 Allogeneic Stem Cell Transplant With a Novel Conditioning Therapy Using Helical Tomotherapy, Melphalan, and Fludarabine in Hematological Malignancies Active, not recruiting NCT00544466 Phase 1, Phase 2 fludarabine phosphate;melphalan
48 A Phase II Study of Cladribine, Cytarabine, and Granulocyte-Colony Stimulating Factor With Fractionated Gemtuzumab Ozogamicin (CLAG-GO) for the Treatment of Patients With Persistent, Relapsed or Refractory Acute Myeloid Leukemia Not yet recruiting NCT04050280 Phase 2 Cladribine, Cytarabine, and Granulocyte-Colony Stimulating Factor with Fractionated Gemtuzumab Ozogamicin (CLAG-GO)
49 A Multi-Center, Open-Label Phase 1b/2 Study of Inotuzumab Ozogamicin and Vincristine Sulfate Liposome in Adult Patients With Relapsed/Refractory B Lineage Acute Lymphoblastic Leukemia (B-ALL) Not yet recruiting NCT03851081 Phase 1, Phase 2 Vincristine Sulfate Liposome
50 A Phase 2 Study of Inotuzumab Ozogamicin (NSC# 772518) in Children and Young Adults With Relapsed or Refractory CD22+ B-Acute Lymphoblastic Leukemia (B-ALL) Suspended NCT02981628 Phase 2

Search NIH Clinical Center for Hepatic Veno-Occlusive Disease

Inferred drug relations via UMLS 71 / NDF-RT 50 :


Antithrombin III, Human
ANTITHROMBIN III,RECOMBINANT

Cochrane evidence based reviews: hepatic veno-occlusive disease

Genetic Tests for Hepatic Veno-Occlusive Disease

Anatomical Context for Hepatic Veno-Occlusive Disease

MalaCards organs/tissues related to Hepatic Veno-Occlusive Disease:

40
Liver, Bone, Bone Marrow, Myeloid, Endothelial, Lung, B Cells

Publications for Hepatic Veno-Occlusive Disease

Articles related to Hepatic Veno-Occlusive Disease:

(show top 50) (show all 2123)
# Title Authors PMID Year
1
The thiopurine methyltransferase genetic polymorphism is associated with thioguanine-related veno-occlusive disease of the liver in children with acute lymphoblastic leukemia. 61 54
17015055 2006
2
Costs of allogeneic hematopoietic stem cell transplantation. 54 61
16858272 2006
3
Control of hepatic veno-occlusive disease with an antithrombin-III concentrate-based therapy. 61 54
16490079 2006
4
Anti-thrombin III in the management of hematopoietic stem-cell transplantation-associated toxicity. 61 54
15113990 2004
5
Role of plasminogen activator inhibitor-1 (PAI-1) levels in the diagnosis of BMT-associated hepatic veno-occlusive disease and monitoring of subsequent therapy with defibrotide (DF). 61 54
12745658 2003
6
Plasminogen activator inhibitor-1 is an independent diagnostic marker as well as severity predictor of hepatic veno-occlusive disease after allogeneic bone marrow transplantation in adults conditioned with busulphan and cyclophosphamide. 61 54
12199790 2002
7
Multiple organ dysfunction syndrome in hematopoietic stem cell transplantation. 61 54
12004245 2002
8
Portal vein thrombosis after hematopoietic cell transplantation: frequency, treatment and outcome. 61 54
11896430 2002
9
Growth factors and hemostasis: differential effects of GM-CSF and G-CSF on coagulation activation--laboratory and clinical evidence. 61 54
11669301 2001
10
Antithrombin treatment of severe hepatic veno-occlusive disease in children with cancer. 61 54
10650858 1999
11
Tissue plasminogen activator (tPA) as therapy for hepatotoxicity following bone marrow transplantation. 54 61
10627640 1999
12
The relevance of plasminogen activator inhibitor 1 (PAI-1) as a marker for the diagnosis of hepatic veno-occlusive disease in patients after bone marrow transplantation. 61 54
10194118 1999
13
Relevance of proteins C and S, antithrombin III, von Willebrand factor, and factor VIII for the development of hepatic veno-occlusive disease in patients undergoing allogeneic bone marrow transplantation: a prospective study. 61 54
9827816 1998
14
Endothelial dysfunction after bone marrow transplantation: increase of soluble thrombomodulin and PAI-1 in patients with multiple transplant-related complications. 61 54
9540759 1998
15
Plasminogen activator inhibitor-1 confirms the diagnosis of hepatic veno-occlusive disease in patients with hyperbilirubinemia after bone marrow transplantation. 61 54
9058743 1997
16
Laboratory markers of veno-occlusive disease in the course of bone marrow and subsequent liver transplantation. 61 54
9052916 1997
17
Protein C, protein S and antithrombin III levels in the course of bone marrow and subsequent liver transplantation due to veno-occlusive disease. 61 54
9438165 1996
18
Recombinant human tissue plasminogen activator for the treatment of severe hepatic veno-occlusive disease in pediatric bone marrow transplant patients. 61 54
8807121 1996
19
Etoposide with/without G-CSF with busulfan and cyclophosphamide as conditioning for bone marrow transplantation. The BMT Team. 61 54
8602625 1996
20
Treatment of veno-occlusive disease of the liver with bolus tissue plasminogen activator and continuous infusion antithrombin III concentrate. 54 61
8704704 1996
21
Successful treatment of veno-occlusive disease with recombinant tissue plasminogen activator in a patient requiring peritoneal dialysis. 61 54
7858540 1994
22
Alteplase for hepatic veno-occlusive disease after bone marrow transplantation. 61 54
1354293 1992
23
Alteplase for hepatic veno-occlusive disease after bone-marrow transplantation. 54 61
1351153 1992
24
Alteplase for hepatic veno-occlusive disease complicating bone-marrow transplantation. 61 54
1349081 1992
25
Ocular hemorrhage secondary to thioguanine-associated veno-occlusive disease in a child with acute lymphoblastic leukemia in delayed intensification. 61
31840375 2019
26
Effects of recombinant thrombomodulin on long-term prognosis after allogeneic hematopoietic stem cell transplantation. 61
31666188 2019
27
Bone marrow transplant with post-transplant cyclophosphamide for recessive dystrophic epidermolysis bullosa expands the related donor pool and permits tolerance of nonhaematopoietic cellular grafts. 61
30843184 2019
28
A Phase 1 Study of Intravenous Busulfan as a Conditioning Regimen for Multiple Myeloma. 61
31619057 2019
29
Acute kidney injury in hematopoietic stem cell transplantation. 61
31524721 2019
30
Publisher Correction: Systematic review of defibrotide studies in the treatment of veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS). 61
30932018 2019
31
Systematic review of defibrotide studies in the treatment of veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS). 61
30804485 2019
32
Diagnosis, grading, and treatment recommendations for children, adolescents, and young adults with sinusoidal obstructive syndrome: an international expert position statement. 61
31818728 2019
33
Pharmacokinetic/Pharmacodynamic Modeling to Support the Re-approval of Gemtuzumab Ozogamicin. 61
31070776 2019
34
Refractory Thrombocytopenia Is a Valid Early Diagnostic Criteria for Hepatic Veno-Occlusive Disease in Children. 61
31756537 2019
35
Successful Treatment of Veno-occlusive Disease, Transplantation-Associated Thrombotic Microangiopathy, and Acute Graft-vs-Host Disease in a Patient with Relapsed Epstein-Barr Hemophagocytic Lymphohistiocytosis After Haploidentical Hematopoietic Stem Cell Transplantation: A Case Report. 61
31711585 2019
36
Secondary hemophagocytic syndrome after autologous hematopoietic cell transplant and immune therapy for neuroblastoma. 61
31407508 2019
37
Efficacy and Safety of Defibrotide for the Treatment of Hepatic Veno-Occlusive Disease after Hematopoietic Stem Cell Transplantation: A Systematic Review and Meta-Analysis. 61
31627217 2019
38
An Autopsy Case of Pulmonary Capillary Hemangiomatosis with an Electron Microscopy Study. 61
31636247 2019
39
Prophylactic, preemptive, and curative treatment for sinusoidal obstruction syndrome/veno-occlusive disease in adult patients: a position statement from an international expert group. 61
31576023 2019
40
Incidence, Predictors, and Outcomes of Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome after Reduced-Intensity Allogeneic Hematopoietic Cell Transplantation. 61
31678537 2019
41
Association between Vitamin D and Risk for Early and Late Post-Transplant Complications. 61
31654787 2019
42
The Role of Interventional Radiology in the Treatment of Acute Thrombosis and Chronic Veno-Occlusive Disease in Children and Adolescents. 61
31706366 2019
43
Computed tomographic and clinical features of pulmonary veno-occlusive disease: raising the radiologist's awareness. 61
31178067 2019
44
Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. 61
31501875 2019
45
Analysis of glutathione S-transferase and cytochrome P450 gene polymorphism in recipients of dose-adjusted busulfan-cyclophosphamide conditioning. 61
31555969 2019
46
Feasibility and Safety of Treosulfan, Melphalan, and Thiotepa-Based Megachemotherapy with Autologous or Allogeneic Stem Cell Transplantation in Heavily Pretreated Children with Relapsed or Refractory Neuroblastoma. 61
31085306 2019
47
The Clinical and Cost Effectiveness of Inotuzumab Ozogamicin for the Treatment of Adult Relapsed or Refractory B-Cell Acute Lymphoblastic Leukaemia: An Evidence Review Group Evaluation of a NICE Single Technology Appraisal. 61
30887470 2019
48
Racial and Ethnic Differences in Pediatric Pulmonary Hypertension: An Analysis of the Pediatric Pulmonary Hypertension Network Registry. 61
31176455 2019
49
Adjuvant recombinant thrombomodulin therapy for hepatopathy induced by vincristine, actinomycin D, and cyclophosphamide in pediatric rhabdomyosarcoma: A case report. 61
31281657 2019
50
Validation of treatment outcomes according to revised severity criteria from European Society for Blood and Marrow Transplantation (EBMT) for sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD). 61
30809031 2019

Variations for Hepatic Veno-Occlusive Disease

Expression for Hepatic Veno-Occlusive Disease

Search GEO for disease gene expression data for Hepatic Veno-Occlusive Disease.

Pathways for Hepatic Veno-Occlusive Disease

GO Terms for Hepatic Veno-Occlusive Disease

Cellular components related to Hepatic Veno-Occlusive Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell surface GO:0009986 9.8 VEGFA THBD PLG PLAT F3 ABCB1
2 extracellular region GO:0005576 9.73 VEGFA THPO SERPINE1 SERPINC1 PLG PLAT
3 collagen-containing extracellular matrix GO:0062023 9.65 SERPINE1 SERPINC1 PLG PLAT F3
4 extracellular space GO:0005615 9.47 VEGFA THPO THBD SERPINE1 SERPINC1 PLG
5 platelet alpha granule lumen GO:0031093 9.46 VEGFA SERPINE1 PLG F8

Biological processes related to Hepatic Veno-Occlusive Disease according to GeneCards Suite gene sharing:

(show all 17)
# Name GO ID Score Top Affiliating Genes
1 angiogenesis GO:0001525 9.84 VEGFA SERPINE1 FGF2 CXCL8
2 cytokine-mediated signaling pathway GO:0019221 9.83 VEGFA FGF2 F3 CXCL8 CSF3
3 positive regulation of protein kinase B signaling GO:0051897 9.78 THPO FGF2 F3 CSF3
4 platelet degranulation GO:0002576 9.71 VEGFA SERPINE1 PLG F8
5 positive regulation of endothelial cell proliferation GO:0001938 9.7 VEGFA FGF2 F3
6 positive regulation of angiogenesis GO:0045766 9.65 VEGFA SERPINE1 FGF2 F3 CXCL8
7 negative regulation of wound healing GO:0061045 9.57 SERPINE1 FGF2
8 negative regulation of blood coagulation GO:0030195 9.56 THBD SERPINE1
9 positive regulation of positive chemotaxis GO:0050927 9.55 VEGFA F3
10 fibrinolysis GO:0042730 9.54 SERPINE1 PLG PLAT
11 stem cell proliferation GO:0072089 9.51 FGF2 ABCB1
12 induction of positive chemotaxis GO:0050930 9.5 VEGFA CXCL8 AZU1
13 negative regulation of cell-cell adhesion mediated by cadherin GO:2000048 9.49 VEGFA PLG
14 trans-synaptic signaling by BDNF, modulating synaptic transmission GO:0099183 9.46 PLG PLAT
15 blood coagulation GO:0007596 9.43 THBD SERPINC1 PLG PLAT F8 F3
16 negative regulation of fibrinolysis GO:0051918 9.33 THBD SERPINE1 PLG
17 hemostasis GO:0007599 9.02 THBD SERPINC1 PLG F8 F3

Molecular functions related to Hepatic Veno-Occlusive Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 signaling receptor binding GO:0005102 9.65 THPO SERPINE1 PLG PLAT HFE
2 heparin binding GO:0008201 9.62 VEGFA SERPINC1 FGF2 AZU1
3 growth factor activity GO:0008083 9.46 VEGFA THPO FGF2 CSF3
4 serine-type endopeptidase activity GO:0004252 9.26 PLG PLAT F3 AZU1
5 cytokine activity GO:0005125 9.02 VEGFA THPO FGF2 CXCL8 CSF3

Sources for Hepatic Veno-Occlusive Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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