MCID: HPT046
MIFTS: 56

Hepatic Veno-Occlusive Disease

Categories: Cardiovascular 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 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
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 hypertension. An important gene associated with Hepatic Veno-Occlusive Disease is SP110 (SP110 Nuclear Body Protein), and among its related pathways/superpathways are Response to elevated platelet cytosolic Ca2+ and Hematopoietic Stem Cells and Lineage-specific Markers. The drugs Busulfan and Fludarabine 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 465)
# Related Disease Score Top Affiliating Genes
1 pulmonary edema 31.2 SERPINC1 CXCL8 CSF3
2 portal hypertension 31.0 VEGFA THPO F3 CXCL8
3 portal vein thrombosis 30.9 SERPINE1 SERPINC1 PLAT
4 antithrombin iii deficiency 30.6 SERPINC1 PLG F3
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 nodular regenerative hyperplasia 30.4 TPMT F3
9 purpura 30.4 THPO THBD SERPINC1 F3
10 mucositis 30.4 VEGFA CSF3 ABCB1
11 hemolytic-uremic syndrome 30.4 THBD SERPINC1 F3 CXCL8
12 endocarditis 30.4 SERPINE1 SERPINC1 PLAT CXCL8
13 toxic shock syndrome 30.3 SERPINC1 F3 CXCL8
14 adult respiratory distress syndrome 30.3 THBD SERPINE1 CXCL8 CSF3
15 engraftment syndrome 30.3 CXCL8 CSF3 ABCB1
16 liver cirrhosis 30.3 THPO SERPINC1 HFE F3
17 castleman disease 30.3 VEGFA CXCL8 CSF3
18 cardiac tamponade 30.3 PLG PLAT F8 F3
19 poems syndrome 30.3 VEGFA FGF2 CSF3
20 thrombophilia due to thrombin defect 30.2 THBD SERPINE1 SERPINC1 PLAT F8 F3
21 pulmonary embolism 30.2 THBD SERPINE1 SERPINC1 PLAT F8 F3
22 exanthem 30.2 VEGFA F3 CXCL8 CSF3
23 thrombosis 29.9 THBD SERPINE1 SERPINC1 PLG PLAT F8
24 childhood acute lymphocytic leukemia 29.9 VEGFA TPMT ABCB1
25 thrombophlebitis 29.8 SERPINE1 SERPINC1 PLG PLAT F8 F3
26 pancytopenia 29.8 TPMT THPO CSF3 CD33
27 antiphospholipid syndrome 29.7 THBD SULT1A3 SERPINE1 SERPINC1 PLAT F3
28 neutropenia 29.7 TPMT THPO CXCL8 CSF3 ABCB1
29 non-alcoholic steatohepatitis 29.7 SERPINE1 HFE CXCL8
30 peripheral nervous system disease 29.6 VEGFA FGF2 CXCL8 CSF3
31 varicose veins 29.6 VEGFA THBD SERPINC1 PLAT HFE FGF2
32 disseminated intravascular coagulation 29.5 THBD SULT1A3 SERPINE1 SERPINC1 PLG PLAT
33 leukemia, chronic myeloid 29.5 VEGFA THPO CSF3 CD33 ABCB1
34 hematologic cancer 29.3 VEGFA THPO CSF3 CD33 ABCB1
35 diarrhea 29.3 THBD CXCL8 CSF3 ABCB1
36 thrombocytopenia 29.2 THPO THBD SERPINE1 SERPINC1 PLAT F3
37 inflammatory bowel disease 29.2 VEGFA TPMT SERPINC1 FGF2 F3 CXCL8
38 vascular disease 29.1 VEGFA THBD SERPINE1 SERPINC1 PLG PLAT
39 pulmonary hypertension 29.1 VEGFA THBD SERPINE1 SERPINC1 PLG PLAT
40 aplastic anemia 28.9 VEGFA TPMT THPO THBD SERPINE1 SERPINC1
41 myelodysplastic syndrome 28.8 VEGFA THPO HFE FGF2 CXCL8 CSF3
42 leukemia, acute myeloid 28.7 VEGFA THPO FGF2 CXCL8 CSF3 CD33
43 systemic lupus erythematosus 28.7 VEGFA THBD SULT1A3 SERPINE1 F3 CXCL8
44 hepatic vascular disease 28.7 VEGFA THPO SP110 SERPINE1 SERPINC1 PLG
45 myeloma, multiple 28.6 VEGFA FGF2 CXCL8 CSF3 CD33 CD22
46 acute leukemia 28.5 TPMT THPO CSF3 CD33 CD22 ABCB1
47 leukemia, acute lymphoblastic 28.5 TPMT THPO SERPINC1 HFE CSF3 CD33
48 hepatic venoocclusive disease with immunodeficiency 12.9
49 pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis 12.8
50 pulmonary hypertension, primary, 1 10.9

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 total bilirubin 58 31 hallmark (90%) Very frequent (99-80%) HP:0003573
9 increased body weight 58 31 hallmark (90%) Very frequent (99-80%) HP:0004324
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 10.24 ABCB1 CD22 CSF3 F3 F8 FGF2
2 homeostasis/metabolism MP:0005376 10.13 ABCB1 F3 F8 FGF2 HFE PLAT
3 immune system MP:0005387 10.07 ABCB1 CD22 CSF3 F3 F8 HFE
4 mortality/aging MP:0010768 9.77 ABCB1 CD22 F3 F8 FGF2 HFE
5 liver/biliary system MP:0005370 9.7 ABCB1 HFE PLG SERPINC1 SERPINE1 THBD
6 reproductive system MP:0005389 9.32 ABCB1 CD22 F8 FGF2 PLAT PLG

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 180)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Busulfan Approved, Investigational Phase 4 55-98-1 2478
2
Fludarabine Approved Phase 4 21679-14-1, 75607-67-9 30751
3
Treosulfan Investigational Phase 4 299-75-2 9296
4 Alkylating Agents Phase 4
5 Antineoplastic Agents, Immunological Phase 4
6 Immunoglobulins Phase 4
7 Antibodies Phase 4
8 Gemtuzumab Phase 4
9
Prednisolone phosphate Approved, Vet_approved Phase 2, Phase 3 302-25-0
10
Hydrocortisone Approved, Vet_approved Phase 3 50-23-7 5754
11
Methylprednisolone Approved, Vet_approved Phase 2, Phase 3 83-43-2 6741
12
leucovorin Approved Phase 3 58-05-9 6006 143
13
Methylprednisolone hemisuccinate Approved Phase 2, Phase 3 2921-57-5
14
Hydrocortisone acetate Approved, Vet_approved Phase 3 50-03-3
15 Prednisolone acetate Approved, Vet_approved Phase 2, Phase 3 52-21-1
16
Prednisolone Approved, Vet_approved Phase 2, Phase 3 50-24-8 5755
17
Cytarabine Approved, Experimental, Investigational Phase 3 147-94-4, 65-46-3 6253
18
Lenograstim Approved, Investigational Phase 2, Phase 3 135968-09-1
19
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
20
Vincristine Approved, Investigational Phase 3 57-22-7, 2068-78-2 5978
21
Pegaspargase Approved, Investigational Phase 3 130167-69-0
22
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
23
Daunorubicin Approved Phase 3 20830-81-3 30323
24
Heparin Approved, Investigational Phase 3 9005-49-6 46507594 772
25
Tinzaparin Approved Phase 3 9041-08-1, 9005-49-6 25244225
26
Dalteparin Approved Phase 3 9005-49-6
27
Doxorubicin Approved, Investigational Phase 3 23214-92-8 31703
28
Mercaptopurine Approved Phase 3 50-44-2 667490
29
Thioguanine Approved Phase 3 154-42-7 2723601
30
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 3 1177-87-3
31
Cobalt Approved, Experimental Phase 3 7440-48-4 104729
32
Dexamethasone Approved, Investigational, Vet_approved Phase 3 50-02-2 5743
33 Ethiodized oil Approved, Investigational Phase 3 8008-53-5
34
Iron Approved, Experimental Phase 3 15438-31-0, 7439-89-6 27284 23925
35
Alprostadil Approved, Investigational Phase 2, Phase 3 745-65-3 149351 5280723
36
Hydroxyurea Approved Phase 3 127-07-1 3657
37
Vidarabine Approved, Investigational Phase 3 24356-66-9 21704 32326
38
Melphalan Approved Phase 3 148-82-3 460612 4053
39
alemtuzumab Approved, Investigational Phase 3 216503-57-0
40
Estradiol Approved, Investigational, Vet_approved Phase 3 50-28-2 5757
41
Norgestimate Approved, Investigational Phase 3 35189-28-7 6540478
42
Moxifloxacin Approved, Investigational Phase 3 354812-41-2, 151096-09-2 152946
43
Ethinyl Estradiol Approved Phase 3 57-63-6 5991
44
Polyestradiol phosphate Approved Phase 3 28014-46-2
45
Folic acid Approved, Nutraceutical, Vet_approved Phase 3 59-30-3 6037
46
Prednisolone hemisuccinate Experimental Phase 2, Phase 3 2920-86-7
47 Anticoagulants Phase 3
48 Fibrinolytic Agents Phase 3
49 Coagulants Phase 3
50 Anti-Inflammatory Agents Phase 3

Interventional clinical trials:

(show top 50) (show all 95)
# 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 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
3 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
4 Allogeneic Stem Cell Transplant for Children and Adolescents With Acute Lymoblastic Leukemia FORUM - Pharmacogenomic Study (add-on Study) Recruiting NCT02670564 Phase 4
5 Using Ultrasound Elastography to Predict Development of Sinusoidal Obstruction Syndrome Active, not recruiting NCT03858530 Phase 4
6 Validating Ultrasound Biomarkers for Hepatic Sinusoidal Obstruction Syndrome in Pediatric Hematopoietic Cell Transplant Patients Withdrawn 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 Active, not 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 Antithrombin-III for Patients With Hepatic Veno-occlusive Diseases Following Hematopoietic Stem Cell Transplantation Unknown status NCT01886248 Phase 2 Freeze-dried Concentrated Human Antithrombin Ⅲ 500 IU
22 Targeted Busulfan, Fludarabine Conditioning Regimen for Hematopoietic Stem Cell Transplantation in Genetic Rare Disease Unknown status NCT02034630 Phase 1, Phase 2 Busulfan
23 Hematopoietic Stem Cell Transplantation With Targeted Busulfan and Fludarabine in Pediatric AML Unknown status NCT01274195 Phase 2 Busulfan
24 Gentuzumab Ozogamicin Berfore Allogeneic Stem Cell Transplantation in Patients With Relapsed CD33+ Acute Myeloid Leukemia Unknown status NCT00460447 Phase 1, Phase 2 Gemtuzumab Ozogamicin
25 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
26 Chemotherapy Toxicity Reduction Via Urea Cycle Support Completed NCT00223730 Phase 2 Citrulline;Placebo
27 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
28 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
29 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
30 Adjunct Targeted Biologic Inhibition in Children With Multivessel Intraluminal Pulmonary Vein Stenosis Completed NCT00891527 Phase 1, Phase 2 Bevacizumab (Avastin) and Imatinib Mesylate (Gleevec)
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 Multicenter Study of Unrelated Umbilical Cord Blood as an Alternate Source of Stem Cells for Transplantation Completed NCT00003913 Phase 2 busulfan;cyclophosphamide;methylprednisolone
33 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
34 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
35 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
36 Unrelated Umbilical Cord Blood As An Alternate Source Of Stem Cells Transplantation Completed NCT00055653 Phase 2 busulfan;cyclophosphamide;cyclosporine;fludarabine phosphate;melphalan;methylprednisolone
37 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
38 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
39 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
40 Inotuzumab Ozogamicin for Children With MRD Positive CD22+ Lymphoblastic Leukemia Recruiting NCT03913559 Phase 2 Inotuzumab ozogamicin;Methotrexate;Hydrocortisone;Cytarabine;Diphenhydramine;Acetaminophen;Methylprednisolone
41 Inotuzumab Ozogamicin Post-Transplant for Acute Lymphocytic Leukemia Recruiting NCT03104491 Phase 1, Phase 2 Inotuzumab Ozogamicin
42 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 Recruiting NCT04050280 Phase 2 Cladribine, Cytarabine, and Granulocyte-Colony Stimulating Factor with Fractionated Gemtuzumab Ozogamicin (CLAG-GO)
43 Targeted Busulfan, Fludarabine, Etoposide Conditioning Regimen for Hematopoietic Stem Cell Transplantation in Childhood and Adolescent Acute Lymphoblastic Leukemia Recruiting NCT02047578 Phase 2 Busulfan
44 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
45 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
46 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
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 Phase II Open Label Study to Assess Efficacy of 5-day Defibrotide Treatment for Hepatic SOS/VOD Not yet recruiting NCT04313036 Phase 2 Defibrotide
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 Use of Defibrotide to Reduce Progression of Acute Respiratory Failure Rate in Patients With COVID-19 Pneumonia Not yet recruiting NCT04335201 Phase 2 Defibrotide Injection

Search NIH Clinical Center for Hepatic Veno-Occlusive Disease

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


Antithrombin III, Human
ANTITHROMBIN III,RECOMBINANT

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 2170)
# 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. 54 61
17015055 2006
2
Costs of allogeneic hematopoietic stem cell transplantation. 61 54
16858272 2006
3
Control of hepatic veno-occlusive disease with an antithrombin-III concentrate-based therapy. 54 61
16490079 2006
4
Anti-thrombin III in the management of hematopoietic stem-cell transplantation-associated toxicity. 54 61
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). 54 61
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. 54 61
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. 54 61
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. 54 61
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. 54 61
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. 54 61
9827816 1998
14
Endothelial dysfunction after bone marrow transplantation: increase of soluble thrombomodulin and PAI-1 in patients with multiple transplant-related complications. 54 61
9540759 1998
15
Plasminogen activator inhibitor-1 confirms the diagnosis of hepatic veno-occlusive disease in patients with hyperbilirubinemia after bone marrow transplantation. 54 61
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. 54 61
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. 54 61
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. 54 61
1349081 1992
25
Retrospective Evaluation of Relationship Between Iron Overload and Transplantation Complications in Pediatric Patient Who Underwent Allogeneic Stem Cell Transplantation Due to Acute Leukemia and Myelodysplastic Syndrome. 61
32427707 2020
26
Molecular Profiling of Vascular Remodeling in Chronic Pulmonary Disease. 61
32275906 2020
27
A novel BMPR2 mutation with widely disparate heritable pulmonary arterial hypertension clinical phenotype. 61
32547734 2020
28
Incidence of Anicteric Veno-Occlusive Disease/Sinusoidal Obstruction Syndrome and Outcomes with Defibrotide following Hematopoietic Cell Transplantation in Adult and Pediatric Patients. 61
32200121 2020
29
Endophlebectomy of the common femoral vein and endovascular iliac vein recanalization for chronic iliofemoral venous occlusion. 61
31932247 2020
30
Carnitine Profile Changes in Pediatric Hematopoietic Stem Cell Transplant: New Role for Carnitine? 61
32032238 2020
31
Pulmonary capillary haemangiomatosis: a distinct entity? 61
32461209 2020
32
Defibrotide inhibits donor leucocyte-endothelial interactions and protects against acute graft-versus-host disease. 61
32519822 2020
33
Reduced-Intensity Conditioning with Busulfan and Fludarabine for Allogeneic Hematopoietic Stem Cell Transplantation in Acute Lymphoblastic Leukemia. 61
32469169 2020
34
Pathologic Hepatic Contrast-Enhanced Ultrasound Pattern in Patients Undergoing Allogeneic Stem Cell Transplantation. 61
32499194 2020
35
Treatment with Intravenous Busulfan, Melphalan, and Etoposide (BuME) followed by Autologous Stem Cell Transplantation in Patients with Non-Hodgkin's Lymphoma: A Multicenter Study from the Consortium for Improving Survival of Lymphoma (CISL). 61
32479690 2020
36
Safety of gemtuzumab ozogamicin as monotherapy or combination therapy in an expanded-access protocol for patients with relapsed or refractory acute myeloid leukemia. 61
32432489 2020
37
Prior Gemtuzumab Ozogamicin Exposure in Adults with Acute Myeloid Leukemia Does Not Increase Hepatic Veno-Occlusive Disease Risk after Allogeneic Hematopoietic Cell Transplantation: A Center for International Blood and Marrow Transplant Research Analysis. 61
31891815 2020
38
Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. 61
31501875 2020
39
Clinical characteristics and survival of Chinese patients diagnosed with pulmonary arterial hypertension who carry BMPR2 or EIF2KAK4 variants. 61
32471403 2020
40
Pulmonary veno-occlusive disease: a probably underdiagnosed cause of pulmonary hypertension in systemic sclerosis. 61
31965379 2020
41
Hepatotoxic pyrrolizidine alkaloids induce DNA damage response in rat liver in a 28-day feeding study. 61
32419051 2020
42
The Speckled Protein (SP) Family: Immunity's Chromatin Readers. 61
32386862 2020
43
Can We Improve Our Diagnostic Accuracy with Ultrasonography in Hepatic Veno-Occlusive Disease? 61
32270740 2020
44
Busulfan clearance does not predict the development of hepatic veno-occlusive disease in patients undergoing hematopoietic stem cell transplantation. 61
32468179 2020
45
Ultrasound in Hepatic Veno-occlusive Disease (HVOD) after Pediatric Hematopoietic Stem Cell Transplantation (HSCT): Comparison of Diagnostic Criteria Including the Pediatric Criteria of European Society for Blood and Marrow Transplantation (EBMT). 61
32131664 2020
46
Introduction of new pediatric EBMT criteria for VOD diagnosis: is it time-saving or money-wasting? : Prospective evaluation of pediatric EBMT criteria for VOD. 61
32398785 2020
47
Case 276: Pulmonary Veno-Occlusive Disease and Pulmonary Capillary Hemangiomatosis Disease. 61
32176598 2020
48
Phase II study of safety and efficacy of BEB (bendamustine, etoposide, and busulfan) conditioning regimen for autologous stem cell transplantation in non-Hodgkin lymphoma. 61
32025840 2020
49
Eating Cheerios on ECMO: The Use of VA ECMO as a Bridge to Lung Transplant in an Infant. 61
32465083 2020
50
Hematopoietic stem cell transplantation in children with Griscelli syndrome type 2: a single-center report on 35 patients. 61
32286505 2020

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 extracellular region GO:0005576 10 VEGFA THPO SERPINE1 SERPINC1 PLG PLAT
2 collagen-containing extracellular matrix GO:0062023 9.72 SERPINE1 SERPINC1 PLG PLAT F3
3 cell surface GO:0009986 9.7 VEGFA THBD PLG PLAT F3 CD22
4 extracellular space GO:0005615 9.44 VEGFA THPO THBD SERPINE1 SERPINC1 PLG
5 platelet alpha granule lumen GO:0031093 9.26 VEGFA SERPINE1 PLG F8

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

(show all 16)
# Name GO ID Score Top Affiliating Genes
1 angiogenesis GO:0001525 9.84 VEGFA SERPINE1 FGF2 CXCL8
2 cytokine-mediated signaling pathway GO:0019221 9.8 VEGFA FGF2 F3 CXCL8 CSF3
3 positive regulation of protein kinase B signaling GO:0051897 9.76 THPO FGF2 F3 CSF3
4 positive regulation of endothelial cell proliferation GO:0001938 9.67 VEGFA FGF2 F3
5 platelet degranulation GO:0002576 9.67 VEGFA SERPINE1 PLG F8
6 positive regulation of angiogenesis GO:0045766 9.65 VEGFA SERPINE1 FGF2 F3 CXCL8
7 induction of positive chemotaxis GO:0050930 9.56 VEGFA CXCL8
8 negative regulation of blood coagulation GO:0030195 9.55 THBD SERPINE1
9 positive regulation of positive chemotaxis GO:0050927 9.52 VEGFA F3
10 negative regulation of cell-cell adhesion mediated by cadherin GO:2000048 9.51 VEGFA PLG
11 fibrinolysis GO:0042730 9.5 SERPINE1 PLG PLAT
12 stem cell proliferation GO:0072089 9.48 FGF2 ABCB1
13 trans-synaptic signaling by BDNF, modulating synaptic transmission GO:0099183 9.43 PLG PLAT
14 blood coagulation GO:0007596 9.43 THBD SERPINC1 PLG PLAT F8 F3
15 negative regulation of fibrinolysis GO:0051918 9.33 THBD SERPINE1 PLG
16 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 growth factor activity GO:0008083 9.46 VEGFA THPO FGF2 CSF3
2 cytokine activity GO:0005125 9.35 VEGFA THPO FGF2 CXCL8 CSF3
3 signaling receptor binding GO:0005102 9.1 THPO SERPINE1 PLG PLAT HFE CD22

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
Content
Loading form....