MCID: EVN001
MIFTS: 45

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 49 55 51 41 69
Autoimmune Hemolytic Anemia and Autoimmune Thrombocytopenia 49 55
Immune Pancytopenia 55
Evan's Syndrome 72
Evan Syndrome 49

Characteristics:

Orphanet epidemiological data:

55
evans syndrome
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe); Age of onset: Adult,Childhood;

Classifications:



Summaries for Evans' Syndrome

NIH Rare Diseases : 49 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 anemia, autoimmune hemolytic and purpura, 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), and among its related pathways/superpathways are IgA-Producing B Cells in the Intestine and Malaria. The drugs Miconazole and Everolimus have been mentioned in the context of this disorder. Affiliated tissues include t cells, thyroid and prostate, and related phenotypes are hematopoietic system and immune system

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 122)
# Related Disease Score Top Affiliating Genes
1 anemia, autoimmune hemolytic 30.1 CD40LG HP
2 purpura 29.7 ADAMTS13 CD40LG
3 spastic paraplegia and evans syndrome 11.9
4 tylosis with esophageal cancer 11.7
5 autoimmune hemolytic anemia-autoimmune thrombocytopenia-primary immunodeficiency syndrome 11.2
6 cataract 31, multiple types 10.3 HP TGFB1
7 thrombotic thrombocytopenic purpura, congenital 10.3 ADAMTS13 HP
8 cryofibrinogenemia 10.2 CD40LG HP
9 geniculate herpes zoster 10.2 CD40LG HP
10 bronchopneumonia 10.2 CD40LG HP
11 nephrosclerosis 10.2 ADAMTS13 TGFB1
12 giardiasis 10.2 CD40LG TGFB1
13 facial paralysis 10.2 CD40LG TGFB1
14 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.2
15 dirofilariasis 10.2 CD40LG HP
16 bilirubin metabolic disorder 10.2 ADAMTS13 HP
17 rheumatic fever 10.2 CD40LG HP
18 pustulosis palmaris et plantaris 10.1 CD40LG TGFB1
19 catastrophic antiphospholipid syndrome 10.1 ADAMTS13 CD40LG
20 norwegian scabies 10.1 CD40LG TGFB1
21 primary thrombocytopenia 10.1 ADAMTS13 CD40LG
22 guillain-barre syndrome 10.1 CD40LG HP
23 idiopathic neutropenia 10.1 CD40LG TGFB1
24 parasitic protozoa infectious disease 10.1 CD40LG HP
25 mixed connective tissue disease 10.1 ADAMTS13 CD40LG
26 blood coagulation disease 10.1 ADAMTS13 CD40LG
27 antiphospholipid syndrome 10.0 ADAMTS13 CD40LG
28 lymphoma 10.0
29 blood platelet disease 10.0 ADAMTS13 CD40LG
30 hemolytic anemia 10.0
31 urinary system disease 10.0 CD40LG TGFB1
32 iga glomerulonephritis 9.9 CD40LG TGFB1
33 pancytopenia 9.9
34 hepatitis 9.9
35 autoimmune disease of blood 9.9 ADAMTS13 CD40LG HP
36 selective immunoglobulin deficiency disease 9.9 CD40LG MAGT1
37 dysgammaglobulinemia 9.9 CD40LG MAGT1
38 immune system disease 9.9 ADAMTS13 CD40LG
39 lymphoproliferative syndrome 2 9.8 CD40LG MAGT1
40 autoimmune hepatitis 9.8
41 common variable immunodeficiency 9.8
42 pure red-cell aplasia 9.8
43 thyroiditis 9.8
44 systemic scleroderma 9.8 CD40LG TGFB1
45 congenital disorder of glycosylation, type in 9.8 HP MAGT1
46 lymphoma, hodgkin, classic 9.7
47 autoimmune lymphoproliferative syndrome 9.7
48 thrombocytopenia 9.7
49 lymphoproliferative syndrome 9.7
50 nocardiosis 9.7

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:

55 31 (show all 13)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 autoimmune hemolytic anemia 55 31 obligate (100%) Obligate (100%) HP:0001890
2 autoimmune thrombocytopenia 55 31 obligate (100%) Obligate (100%) HP:0001973
3 petechiae 55 31 hallmark (90%) Very frequent (99-80%) HP:0000967
4 autoimmune neutropenia 55 31 hallmark (90%) Very frequent (99-80%) HP:0001904
5 epistaxis 55 31 frequent (33%) Frequent (79-30%) HP:0000421
6 jaundice 55 31 frequent (33%) Frequent (79-30%) HP:0000952
7 bruising susceptibility 55 31 frequent (33%) Frequent (79-30%) HP:0000978
8 pallor 55 31 frequent (33%) Frequent (79-30%) HP:0000980
9 lethargy 55 31 frequent (33%) Frequent (79-30%) HP:0001254
10 muscle weakness 55 31 frequent (33%) Frequent (79-30%) HP:0001324
11 dyspnea 55 31 frequent (33%) Frequent (79-30%) HP:0002094
12 fatigue 55 31 frequent (33%) Frequent (79-30%) HP:0012378
13 syncope 55 31 occasional (7.5%) Occasional (29-5%) HP:0001279

MGI Mouse Phenotypes related to Evans' Syndrome:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.43 ADAMTS13 CD40LG HP LRBA TGFB1 TPP2
2 immune system MP:0005387 9.1 ADAMTS13 CD40LG HP LRBA TGFB1 TPP2

Drugs & Therapeutics for Evans' Syndrome

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

(show all 9)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Miconazole Approved, Investigational, Vet_approved Phase 1, Phase 2 22916-47-8 4189
2
Everolimus Approved Phase 1, Phase 2 159351-69-6 6442177
3
Sirolimus Approved, Investigational Phase 1, Phase 2 53123-88-9 46835353 6436030 5284616
4 Anti-Bacterial Agents Phase 1, Phase 2
5 Anti-Infective Agents Phase 1, Phase 2
6 Antifungal Agents Phase 1, Phase 2
7 Immunosuppressive Agents Phase 1, Phase 2
8 Antibiotics, Antitubercular Phase 1, Phase 2
9
Cyclophosphamide Approved, Investigational Phase 1 50-18-0, 6055-19-2 2907

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Sirolimus for Autoimmune Disease of Blood Cells Active, not recruiting NCT00392951 Phase 1, Phase 2 sirolimus
2 Treatment of Autoimmune Thrombocytopenia (AITP) Completed NCT00001630 Phase 1

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:

38
T Cells, Thyroid, Prostate, B Cells, Pituitary, Pancreas, Bone

Publications for Evans' Syndrome

Articles related to Evans' Syndrome:

(show top 50) (show all 68)
# Title Authors Year
1
Association of autoimmune hepatitis type 1 in a child with Evans syndrome. ( 28878866 )
2017
2
Persistent Fever in Medical Intensive Care Unit: Evans Syndrome Versus Actinomycotic Osteomyelitis? ( 28092284 )
2017
3
Evans Syndrome Secondary to Common Variable Immune Deficiency. ( 26950085 )
2016
4
Evans syndrome with non-ST segment elevation myocardial infarction complicated by hemopericardium. ( 27751316 )
2016
5
Evans syndrome after autologous peripheral blood stem cell transplantation for recurrent Hodgkin lymphoma. ( 27577012 )
2016
6
Unusual co-occurrence of Evans syndrome and pure red cell aplasia in elderly patient with prostate cancer. ( 27465154 )
2016
7
Successful treatment with rituximab and romiplostim for thrombocytopenia associated with WaldenstrAPm's macroglobulinemia initially presenting as Evans syndrome. ( 25876786 )
2015
8
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
9
A Case of a TSH-secreting Pituitary Adenoma Associated with Evans' Syndrome. ( 26150183 )
2015
10
Early-onset Evans syndrome, immunodeficiency, and premature immunosenescence associated with tripeptidyl-peptidase II deficiency. ( 25414442 )
2015
11
Profound autoimmune hemolysis and Evans syndrome in two asplenic patients with babesiosis. ( 25354478 )
2014
12
A case of Evans' syndrome following influenza vaccine. ( 22796025 )
2013
13
CD56+ angioimmunoblastic T-cell lymphoma with evans syndrome : a case report and review of the literature. ( 23801132 )
2013
14
Hashimoto's thyroiditis associated Evans syndrome: A rare case report on the clustered autoimmune disease triad. ( 23961497 )
2013
15
Refractory thrombocytopenia responds to octreotide treatment in a case of evans syndrome with gastric neuroendocrine tumor. ( 23984123 )
2013
16
Evaluation of children with chronic immune thrombocytopenic purpura and Evans syndrome treated with rituximab. ( 22815318 )
2013
17
Autoimmune hepatitis-primary biliary cirrhosis overlap syndrome concomitant with immune hemolytic anemia and immune thrombocytopenic purpura (Evans syndrome). ( 23273499 )
2013
18
Dermatomyositis associated with generalized subcutaneous edema and Evans syndrome. ( 22030016 )
2012
19
Evans syndrome with cytomegalovirus infection followed by emerging peripheral T-cell lymphoma. ( 21437588 )
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
Evans syndrome following long-standing Hashimoto's thyroiditis and successful treatment with rituximab. ( 22259635 )
2011
22
Glucocorticoid-resistant Evans' syndrome successfully controlled with low-dose cyclosporine. ( 21961487 )
2011
23
Successful control of steroid-intolerant Evans' syndrome associated with allogeneic peripheral blood hematopoietic stem cell transplant by rituximab. ( 21275632 )
2011
24
Successful rituximab therapy in refractory autoimmune hepatitis and Evans syndrome. ( 22446656 )
2011
25
Rituximab resistant evans syndrome and autoimmunity in Schimke immuno-osseous dysplasia. ( 21914180 )
2011
26
Massive hemoperitoneum with splenic infarction in Evans syndrome. ( 21088621 )
2011
27
Successful remission of Evans syndrome associated with Graves' disease by using propylthiouracil monotherapy. ( 21422690 )
2011
28
Evans' syndrome in pregnancy: a systematic literature review and two new cases. ( 20031296 )
2010
29
Identifying autoimmune lymphoproliferative syndrome in children with Evans syndrome: a multi-institutional study. ( 20068224 )
2010
30
Howel-Evans syndrome: a variant of ectodermal dysplasia. ( 20486456 )
2010
31
Visceral leishmaniasis in a patient with common variable immunodeficiency and evans syndrome: clinical remarks. ( 19579705 )
2009
32
Complications of Evans' syndrome in an infant with hereditary spherocytosis: a case report. ( 19740448 )
2009
33
Treatment of Evans' syndrome with human intravenous immunoglobulin and leflunomide in a diabetic dog. ( 19411652 )
2009
34
Splenic marginal zone lymphoma with Evans' syndrome, autoimmunity, and peripheral gamma/delta T cells. ( 18665362 )
2009
35
Amyloidosis, Evans syndrome and management options of lymphoplasmacytic lymphoma. ( 19291514 )
2009
36
Primary cutaneous nocardiosis in a patient with Evans' Syndrome. ( 19661017 )
2009
37
Pseudohypoparathyroidism Ia with Evans syndrome. ( 18799944 )
2008
38
Common variable immunodeficiency and Evans syndrome complicated by autoimmune hemolysis due to anti-Jka auto-antibodies. ( 18452064 )
2008
39
Consolidation treatment with rituximab induces complete and persistent remission of mixed type Evans syndrome. ( 18469554 )
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
A case of pure red cell aplasia complicated by Evans syndrome. ( 17694270 )
2007
42
Autoimmune hemolytic anemias, Evans' syndromes, and pure red cell aplasia in non-Hodgkin lymphomas. ( 17577777 )
2007
43
Increased lymphocyte Fas expression and high incidence of common variable immunodeficiency disorder in childhood Evans' syndrome. ( 17936685 )
2007
44
Tubercular adenitis and Evans' syndrome. ( 16539285 )
2006
45
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
46
A 20-year-old woman with Hashimoto's thyroiditis and Evans' syndrome. ( 16807996 )
2006
47
Successful treatment of a patient with mixed warm and cold antibody mediated Evans syndrome and glucose intolerance. ( 15704210 )
2005
48
Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS). ( 15542578 )
2005
49
Pegylated interferon alpha-2a-associated life-threatening Evans' syndrome in a patient with chronic hepatitis C. ( 16230187 )
2005
50
Acute renal failure in a young woman with Fisher-Evans' syndrome. ( 15593044 )
2004

Variations for Evans' Syndrome

Expression for Evans' Syndrome

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

Pathways for Evans' Syndrome

Pathways related to Evans' Syndrome according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 10.78 CD40LG TGFB1
2 10.31 CD40LG TGFB1

GO Terms for Evans' Syndrome

Sources for Evans' Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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