MCID: EVN001
MIFTS: 41

Evans' Syndrome

Categories: Rare diseases, Blood diseases, Immune diseases

Aliases & Classifications for Evans' Syndrome

MalaCards integrated aliases for Evans' Syndrome:

Name: Evans' Syndrome 12 14
Evans Syndrome 50 56 52 42 69
Autoimmune Hemolytic Anemia and Autoimmune Thrombocytopenia 50 56
Immune Pancytopenia 56
Evan Syndrome 50

Characteristics:

Orphanet epidemiological data:

56
evans syndrome
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe); Age of onset: Adult,Childhood;

Classifications:



Summaries for Evans' Syndrome

NIH Rare Diseases : 50 evans syndrome is a very rare autoimmune disorder in which the immune system destroys the body's red blood cells, white blood cells and/or platelets. affected people often experience thrombocytopenia (too few platelets) and coombs' positive hemolytic anemia (premature destruction of red blood cells). signs and symptoms may include purpura, paleness, fatigue, and light-headedness. the exact cause of this condition is unknown. the best treatment options for evans syndrome depend on many factors, including the severity of the condition; the signs and symptoms present; and each person's response to certain therapies. last updated: 5/18/2016

MalaCards based summary : Evans' Syndrome, also known as evans syndrome, is related to evans syndrome with immunodeficiency and premature immunosenescence and tylosis with esophageal cancer, and has symptoms including autoimmune hemolytic anemia, autoimmune thrombocytopenia and petechiae. An important gene associated with Evans' Syndrome is ADAMTS13 (ADAM Metallopeptidase With Thrombospondin Type 1 Motif 13). The drugs Acetylcholine and Peripheral Nervous System Agents have been mentioned in the context of this disorder. Affiliated tissues include ovary, brain and lung.

Wikipedia : 72 Evans syndrome is an autoimmune disease in which an individual\'s antibodies attack their own red blood... more...

Related Diseases for Evans' Syndrome

Diseases related to Evans' Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 111)
id Related Disease Score Top Affiliating Genes
1 evans syndrome with immunodeficiency and premature immunosenescence 11.8
2 tylosis with esophageal cancer 11.1
3 autoimmune hemolytic anemia-autoimmune thrombocytopenia-primary immunodeficiency syndrome 10.8
4 porphyria, acute hepatic 10.5 ADAMTS13 HP
5 thumb stiff brachydactyly mental retardation 10.4 ADAMTS13 HP
6 cryptorchidism arachnodactyly mental retardation 10.4 CD40LG HP
7 kidney carcinoma in situ 10.3 CD40LG HP
8 pyelitis 10.3 ADAMTS13 HP
9 shigellosis 10.3 CD40LG HP
10 malignant hypertension 10.3 CD40LG HP
11 cavernous lymphangioma 10.3 ADAMTS13 CD40LG
12 spastic entropion 10.3 CD40LG HP
13 retromolar area cancer 10.3 ADAMTS13 CD40LG
14 endogenous depression 10.3 CD40LG HP
15 ovarian endometrioid cystadenoma 10.3 CD40LG HP
16 choreatic disease 10.2 CD40LG HP
17 extrahepatic bile duct adenocarcinoma 10.2 ADAMTS13 CD40LG
18 ocular hyperemia 10.2 ADAMTS13 CD40LG
19 acute interstitial pneumonia 10.2 CD40LG HP
20 cd40 ligand deficiency 10.2 CD3G CD40LG
21 agammaglobulinemia, microcephaly, and severe dermatitis 10.2 CD3G CD40LG
22 perichondritis of auricle 10.1 ADAMTS13 CD40LG
23 myeloproliferative neoplasm 10.1 ADAMTS13 CD40LG
24 heart fibrosarcoma 10.1 CD40LG MAGT1
25 gestational diabetes 10.1 CD40LG MAGT1
26 mixed germ cell-sex cord neoplasm 10.1 ADAMTS13 CD40LG
27 acute proliferative glomerulonephritis 10.1 ADAMTS13 HP
28 hemolytic anemia due to triosephosphate isomerase deficiency 10.1 CD40LG MAGT1
29 partial sensory epilepsy 10.0 ADAMTS13 CD40LG
30 lymphoma 10.0
31 autonomic peripheral neuropathy 9.9 ADAMTS13 CD40LG HP
32 childhood cerebellar astrocytic neoplasm 9.9 CD3G CD40LG
33 hemolytic anemia 9.9
34 pancytopenia 9.8
35 hepatitis 9.8
36 autoimmune hemolytic anemia 9.8
37 chronic progressive external ophthalmoplegia 9.8 ADAMTS13 CD40LG
38 autoimmune hepatitis 9.8
39 common variable immunodeficiency 9.8
40 pure red-cell aplasia 9.8
41 purpura 9.8
42 thyroiditis 9.8
43 childhood testicular mixed germ cell tumor 9.7 CD3G MAGT1
44 hodgkin lymphoma 9.7
45 autoimmune lymphoproliferative syndrome 9.7
46 thrombocytopenia 9.7
47 lymphoproliferative syndrome 9.7
48 nocardiosis 9.7
49 adenoma 9.7
50 lacrimal system cancer 9.6 ADAMTS13 CD40LG

Graphical network of the top 20 diseases related to Evans' Syndrome:



Diseases related to Evans' Syndrome

Symptoms & Phenotypes for Evans' Syndrome

Human phenotypes related to Evans' Syndrome:

56 32 (show all 13)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 autoimmune hemolytic anemia 56 32 obligate (100%) Obligate (100%) HP:0001890
2 autoimmune thrombocytopenia 56 32 obligate (100%) Obligate (100%) HP:0001973
3 petechiae 56 32 hallmark (90%) Very frequent (99-80%) HP:0000967
4 autoimmune neutropenia 56 32 hallmark (90%) Very frequent (99-80%) HP:0001904
5 epistaxis 56 32 frequent (33%) Frequent (79-30%) HP:0000421
6 jaundice 56 32 frequent (33%) Frequent (79-30%) HP:0000952
7 bruising susceptibility 56 32 frequent (33%) Frequent (79-30%) HP:0000978
8 pallor 56 32 frequent (33%) Frequent (79-30%) HP:0000980
9 lethargy 56 32 frequent (33%) Frequent (79-30%) HP:0001254
10 muscle weakness 56 32 frequent (33%) Frequent (79-30%) HP:0001324
11 dyspnea 56 32 frequent (33%) Frequent (79-30%) HP:0002094
12 fatigue 56 32 frequent (33%) Frequent (79-30%) HP:0012378
13 syncope 56 32 occasional (7.5%) Occasional (29-5%) HP:0001279

Drugs & Therapeutics for Evans' Syndrome

Drugs for Evans' Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 100)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Acetylcholine Approved Phase 4 51-84-3 187
2 Peripheral Nervous System Agents Phase 4,Early Phase 1
3 abobotulinumtoxinA Phase 4
4 Botulinum Toxins Phase 4
5 Botulinum Toxins, Type A Phase 4
6 Cholinergic Agents Phase 4
7 Neuromuscular Agents Phase 4
8 Neurotransmitter Agents Phase 4
9 onabotulinumtoxinA Phase 4
10
Estradiol Approved, Investigational, Vet_approved Phase 3 50-28-2 5757
11
Ethambutol Approved Phase 3 74-55-5 3279 14052
12
Ethinyl Estradiol Approved Phase 3 57-63-6 5991
13
Isoniazid Approved Phase 3 54-85-3 3767
14
Moxifloxacin Approved, Investigational Phase 3 354812-41-2, 151096-09-2 152946
15
Norgestimate Approved Phase 3 35189-28-7 6540478
16
Pyrazinamide Approved Phase 3 98-96-4 1046
17
Rifampin Approved Phase 3 13292-46-1 5458213 5381226
18 Anti-Bacterial Agents Phase 3,Phase 1,Phase 2
19 Antibiotics, Antitubercular Phase 3,Phase 1,Phase 2
20 Anti-Infective Agents Phase 3,Phase 2,Phase 1
21 Antimetabolites Phase 3
22 Antitubercular Agents Phase 3
23 Contraceptive Agents Phase 3
24 Contraceptives, Oral Phase 3
25 Contraceptives, Oral, Combined Phase 3
26 Cytochrome P-450 CYP3A Inducers Phase 3
27 Estradiol 17 beta-cypionate Phase 3
28 Estradiol 3-benzoate Phase 3
29 Estradiol valerate Phase 3 979-32-8
30 Fluoroquinolones Phase 3
31 Hypolipidemic Agents Phase 3
32 Lipid Regulating Agents Phase 3
33 Norgestimate, ethinyl estradiol drug combination Phase 3
34 Nucleic Acid Synthesis Inhibitors Phase 3
35 Polyestradiol phosphate Phase 3
36 Topoisomerase Inhibitors Phase 3
37
Loteprednol Approved Phase 2 82034-46-6, 129260-79-3 444025 9865442
38
Everolimus Approved Phase 1, Phase 2 159351-69-6 6442177
39
Miconazole Approved, Investigational, Vet_approved Phase 1, Phase 2 22916-47-8 4189
40
Sirolimus Approved, Investigational Phase 1, Phase 2 53123-88-9 5284616 6436030 46835353
41 Pharmaceutical Solutions Phase 2
42 Anti-Allergic Agents Phase 2
43 Antimalarials Phase 2
44 Antiparasitic Agents Phase 2
45 Antiprotozoal Agents Phase 2
46 Renal Agents Phase 2
47 Sulfalene Phase 2
48 Antifungal Agents Phase 1, Phase 2
49 Immunosuppressive Agents Phase 1, Phase 2
50 Acidophilus Nutraceutical Phase 2

Interventional clinical trials:

(show all 27)

id Name Status NCT ID Phase Drugs
1 Intravesicular Onabotulinumtoxin A in Interstitial Cystitis Recruiting NCT02297100 Phase 4 Onabotulinumtoxin A
2 Rilonacept for Treatment of Cryopyrin-Associated Periodic Syndromes (CAPS) Completed NCT00288704 Phase 3 rilonacept 160 mg;Placebo;rilonacept 160 mg
3 Controlled Comparison of Two Moxifloxacin Containing Treatment Shortening Regimens in Pulmonary Tuberculosis Completed NCT00864383 Phase 3 Moxifloxacin, Ethambutol, Isoniazid, Pyrazinamide & Rifampicin
4 Using Human Menstrual Blood Cells to Treat Acute Lung Injury Caused by H7N9 Bird Flu Virus Infection Unknown status NCT02095444 Phase 1, Phase 2 Menstrual blood stem cells
5 Acute Human Study: StimRouter for Peripheral Nerve Stimulation of Discrete Peripheral Nerves Completed NCT00665132 Phase 1, Phase 2
6 Hyperbaric Oxygen Therapy (HBO2) for Persistent Post-concussive Symptoms After Mild Traumatic Brain Injury (mTBI) Completed NCT01306968 Phase 2 hyperbaric oxygen
7 Safety and Efficacy of KPI-121 in Subjects With Dry Eye Disease Completed NCT02188160 Phase 2 KPI-121;Placebo
8 Evaluation of the Efficacy of Two Probiotic Strains for Irritable Bowel Syndrome Recruiting NCT02213172 Phase 2
9 Sirolimus for Autoimmune Disease of Blood Cells Active, not recruiting NCT00392951 Phase 1, Phase 2 sirolimus
10 Treatment of Autoimmune Thrombocytopenia (AITP) Completed NCT00001630 Phase 1
11 Higher Protein Diet and Exercise for Optimal Weight Loss in Elderly Women Completed NCT00912210 Phase 1
12 A Multicenter, Randomized, Placebo-Controlled, Double-Blinded, Phase I Trial to Evaluate the Safety and Immunogenicity of Live Recombinant Canarypox ALVAC-HIV vCP205 Combined With GM-CSF in Healthy, HIV-1 Uninfected Volunteers Completed NCT00001090 Phase 1 Sargramostim
13 The Role of Lipoaspirate Injection in the Treatment of Diabetic Lower Extremity Wounds and Venous Stasis Ulcers Unknown status NCT00815217
14 Effect of a High-Protein Diet and/or High-Intensity Training on Metabolic Syndrome Completed NCT01622634
15 Iyengar Yoga for Young People With Irritable Bowel Syndrome Completed NCT01107977
16 Randomized Study of Decreased Hyperinsulinemia on the Ovulatory Response to Clomiphene Citrate in Women With Polycystic Ovary Syndrome Completed NCT00005104 clomiphene citrate;metformin
17 Randomized Study of the Effect of Decreased Hyperinsulinemia on the Ovulatory Response to Clomiphene Citrate in Obese Women With Polycystic Ovary Syndrome Completed NCT00005654 clomiphene citrate;metformin
18 Vitamin D-related Genes and Metabolic Disorders Completed NCT03279432
19 New Markers to Measure Clotting in Patients With the Obstructive Sleep Apnoea Hypopnoea Syndrome Completed NCT01525160
20 Exercise-Induced Epigenetic Modifications in Obese Aging Women Completed NCT01977885
21 mTBI Mechanisms of Action of HBO2 for Persistent Post-Concussive Symptoms Completed NCT01611194 Hyperbaric oxygen (HBO2) at 1.5 atms;Sham control 1.2 atms
22 Study of Effects of Sutures on Results of Palate Surgery for Obstructive Sleep Apnea Completed NCT00205543
23 Rivaroxaban ACS Specialist Cohort Event Monitoring Study Recruiting NCT02673437
24 Remifentanil in Adults With OSA Recruiting NCT02898792 Early Phase 1 Targeted infusion of remifentanil
25 Patients Treated for Wiskott-Aldrich Syndrome (WAS) Since 1990 Recruiting NCT02064933
26 Natural History Study of SCID Disorders Recruiting NCT01186913
27 Normative Datasets for Assessments Planned for Mild Traumatic Brain Injury (NORMAL) Active, not recruiting NCT01925963

Search NIH Clinical Center for Evans' Syndrome

Cochrane evidence based reviews: evans syndrome

Genetic Tests for Evans' Syndrome

Anatomical Context for Evans' Syndrome

MalaCards organs/tissues related to Evans' Syndrome:

39
Ovary, Brain, Lung, Bone, Eye, T Cells, Thyroid

Publications for Evans' Syndrome

Articles related to Evans' Syndrome:

(show top 50) (show all 68)
id Title Authors Year
1
Persistent Fever in Medical Intensive Care Unit: Evans Syndrome Versus Actinomycotic Osteomyelitis? ( 28092284 )
2017
2
Association of autoimmune hepatitis type 1 in a child with Evans syndrome. ( 28878866 )
2017
3
Evans syndrome with non-ST segment elevation myocardial infarction complicated by hemopericardium. ( 27751316 )
2016
4
Evans syndrome after autologous peripheral blood stem cell transplantation for recurrent Hodgkin lymphoma. ( 27577012 )
2016
5
Evans Syndrome Secondary to Common Variable Immune Deficiency. ( 26950085 )
2016
6
Unusual co-occurrence of Evans syndrome and pure red cell aplasia in elderly patient with prostate cancer. ( 27465154 )
2016
7
Early-onset Evans syndrome, immunodeficiency, and premature immunosenescence associated with tripeptidyl-peptidase II deficiency. ( 25414442 )
2015
8
Successful treatment with rituximab and romiplostim for thrombocytopenia associated with WaldenstrAPm's macroglobulinemia initially presenting as Evans syndrome. ( 25876786 )
2015
9
Disseminated intravascular coagulation and immune hemolytic anemia, possibly evans syndrome, after oxaliplatin and bevacizumab infusion for metastatic colon adenocarcinoma: a case report and literature review. ( 25600448 )
2015
10
A Case of a TSH-secreting Pituitary Adenoma Associated with Evans' Syndrome. ( 26150183 )
2015
11
Profound autoimmune hemolysis and Evans syndrome in two asplenic patients with babesiosis. ( 25354478 )
2014
12
Hashimoto's thyroiditis associated Evans syndrome: A rare case report on the clustered autoimmune disease triad. ( 23961497 )
2013
13
Refractory thrombocytopenia responds to octreotide treatment in a case of evans syndrome with gastric neuroendocrine tumor. ( 23984123 )
2013
14
Evaluation of children with chronic immune thrombocytopenic purpura and Evans syndrome treated with rituximab. ( 22815318 )
2013
15
CD56+ angioimmunoblastic T-cell lymphoma with evans syndrome : a case report and review of the literature. ( 23801132 )
2013
16
A case of Evans' syndrome following influenza vaccine. ( 22796025 )
2013
17
Autoimmune hepatitis-primary biliary cirrhosis overlap syndrome concomitant with immune hemolytic anemia and immune thrombocytopenic purpura (Evans syndrome). ( 23273499 )
2013
18
Evans syndrome with cytomegalovirus infection followed by emerging peripheral T-cell lymphoma. ( 21437588 )
2012
19
Dermatomyositis associated with generalized subcutaneous edema and Evans syndrome. ( 22030016 )
2012
20
Successful allogeneic cord blood transplantation in a patient with Evans syndrome leads to correction of hereditary angioedema type I as secondary effect. ( 22327128 )
2012
21
Rituximab resistant evans syndrome and autoimmunity in Schimke immuno-osseous dysplasia. ( 21914180 )
2011
22
Successful control of steroid-intolerant Evans' syndrome associated with allogeneic peripheral blood hematopoietic stem cell transplant by rituximab. ( 21275632 )
2011
23
Glucocorticoid-resistant Evans' syndrome successfully controlled with low-dose cyclosporine. ( 21961487 )
2011
24
Successful rituximab therapy in refractory autoimmune hepatitis and Evans syndrome. ( 22446656 )
2011
25
Massive hemoperitoneum with splenic infarction in Evans syndrome. ( 21088621 )
2011
26
Evans syndrome following long-standing Hashimoto's thyroiditis and successful treatment with rituximab. ( 22259635 )
2011
27
Successful remission of Evans syndrome associated with Graves' disease by using propylthiouracil monotherapy. ( 21422690 )
2011
28
Identifying autoimmune lymphoproliferative syndrome in children with Evans syndrome: a multi-institutional study. ( 20068224 )
2010
29
Howel-Evans syndrome: a variant of ectodermal dysplasia. ( 20486456 )
2010
30
Evans' syndrome in pregnancy: a systematic literature review and two new cases. ( 20031296 )
2010
31
Amyloidosis, Evans syndrome and management options of lymphoplasmacytic lymphoma. ( 19291514 )
2009
32
Treatment of Evans' syndrome with human intravenous immunoglobulin and leflunomide in a diabetic dog. ( 19411652 )
2009
33
Primary cutaneous nocardiosis in a patient with Evans' Syndrome. ( 19661017 )
2009
34
Complications of Evans' syndrome in an infant with hereditary spherocytosis: a case report. ( 19740448 )
2009
35
Visceral leishmaniasis in a patient with common variable immunodeficiency and evans syndrome: clinical remarks. ( 19579705 )
2009
36
Splenic marginal zone lymphoma with Evans' syndrome, autoimmunity, and peripheral gamma/delta T cells. ( 18665362 )
2009
37
Common variable immunodeficiency and Evans syndrome complicated by autoimmune hemolysis due to anti-Jka auto-antibodies. ( 18452064 )
2008
38
Consolidation treatment with rituximab induces complete and persistent remission of mixed type Evans syndrome. ( 18469554 )
2008
39
Pseudohypoparathyroidism Ia with Evans syndrome. ( 18799944 )
2008
40
A dose-escalation study of rituximab for treatment of systemic lupus erythematosus and Evans' syndrome: immunological analysis of B cells, T cells and cytokines. ( 18397955 )
2008
41
Increased lymphocyte Fas expression and high incidence of common variable immunodeficiency disorder in childhood Evans' syndrome. ( 17936685 )
2007
42
Autoimmune hemolytic anemias, Evans' syndromes, and pure red cell aplasia in non-Hodgkin lymphomas. ( 17577777 )
2007
43
A case of pure red cell aplasia complicated by Evans syndrome. ( 17694270 )
2007
44
Development of mixed-type autoimmune hemolytic anemia and Evans' syndrome following chicken pox infection in a case of low-titer cold agglutinin disease. ( 17050195 )
2006
45
A 20-year-old woman with Hashimoto's thyroiditis and Evans' syndrome. ( 16807996 )
2006
46
Tubercular adenitis and Evans' syndrome. ( 16539285 )
2006
47
Pegylated interferon alpha-2a-associated life-threatening Evans' syndrome in a patient with chronic hepatitis C. ( 16230187 )
2005
48
Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS). ( 15542578 )
2005
49
Successful treatment of a patient with mixed warm and cold antibody mediated Evans syndrome and glucose intolerance. ( 15704210 )
2005
50
Evans' syndrome following autologous peripheral blood stem cell transplantation for non-Hodgkin's lymphoma. ( 15279667 )
2004

Variations for Evans' Syndrome

Expression for Evans' Syndrome

Search GEO for disease gene expression data for Evans' Syndrome.

Pathways for Evans' Syndrome

GO Terms for Evans' Syndrome

Biological processes related to Evans' Syndrome according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 T cell costimulation GO:0031295 8.62 CD3G CD40LG

Sources for Evans' Syndrome

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10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
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34 ICD10 via Orphanet
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52 Novoseek
54 OMIM
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59 PubMed
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65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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