MCID: EVN001
MIFTS: 47

Evans' Syndrome

Categories: Rare diseases, Blood diseases, Immune diseases

Aliases & Classifications for Evans' Syndrome

MalaCards integrated aliases for Evans' Syndrome:

Name: Evans' Syndrome 12 15
Evans Syndrome 53 59 55 44 73
Autoimmune Hemolytic Anemia and Autoimmune Thrombocytopenia 53 59
Immune Pancytopenia 59
Evan's Syndrome 76
Evan Syndrome 53

Characteristics:

Orphanet epidemiological data:

59
evans syndrome
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe); Age of onset: Adult,Childhood;

Classifications:



Summaries for Evans' Syndrome

NIH Rare Diseases : 53 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.

MalaCards based summary : Evans' Syndrome, also known as evans syndrome, is related to anemia, autoimmune hemolytic and purpura. An important gene associated with Evans' Syndrome is TPP2 (Tripeptidyl Peptidase 2), and among its related pathways/superpathways is Malaria. The drugs Miconazole and Everolimus have been mentioned in the context of this disorder. Affiliated tissues include t cells, thyroid and bone, and related phenotypes are autoimmune hemolytic anemia and autoimmune thrombocytopenia

Wikipedia : 76 Evans syndrome is an autoimmune disease in which an individual\'s immune system attacks their own red... 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 29.6 CD40LG HP
2 purpura 28.9 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 papillon-lefevre syndrome 10.9
7 cataract 31, multiple types 10.2 HP TGFB1
8 thrombotic thrombocytopenic purpura, congenital 10.1 ADAMTS13 HP
9 lymphoma 10.0
10 hemolytic anemia 10.0
11 bilirubin metabolic disorder 10.0 ADAMTS13 HP
12 nephrosclerosis 10.0 ADAMTS13 TGFB1
13 hepatitis 10.0
14 pancytopenia 9.9
15 autoimmune hepatitis 9.9
16 cryofibrinogenemia 9.9 CD40LG HP
17 geniculate herpes zoster 9.9 CD40LG HP
18 bronchopneumonia 9.9 CD40LG HP
19 common variable immunodeficiency 9.8
20 pure red-cell aplasia 9.8
21 thyroiditis 9.8
22 dirofilariasis 9.8 CD40LG HP
23 rheumatic fever 9.8 CD40LG HP
24 giardiasis 9.8 CD40LG TGFB1
25 facial paralysis 9.8 CD40LG TGFB1
26 pustulosis palmaris et plantaris 9.8 CD40LG TGFB1
27 guillain-barre syndrome 9.8 CD40LG HP
28 systemic lupus erythematosus 9.8
29 lymphoma, hodgkin, classic 9.8
30 myelofibrosis 9.8
31 autoimmune lymphoproliferative syndrome 9.8
32 thrombocytopenia 9.8
33 lymphoproliferative syndrome 9.8
34 nocardiosis 9.8
35 adenoma 9.8
36 lupus erythematosus 9.8
37 norwegian scabies 9.8 CD40LG TGFB1
38 parasitic protozoa infectious disease 9.7 CD40LG HP
39 idiopathic neutropenia 9.7 CD40LG TGFB1
40 catastrophic antiphospholipid syndrome 9.7 ADAMTS13 CD40LG
41 primary thrombocytopenia 9.7 ADAMTS13 CD40LG
42 digeorge syndrome 9.6
43 b-cell lymphomas 9.6
44 autoimmune disease 9.6
45 biliary cirrhosis, primary, 1 9.6
46 prostate cancer 9.6
47 thrombocytopenic purpura, autoimmune 9.6
48 immunodeficiency with hyper-igm, type 1 9.6
49 lymphoma, non-hodgkin, familial 9.6
50 autoimmune disease 1 9.6

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:

59 32 (show all 13)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 autoimmune hemolytic anemia 59 32 obligate (100%) Obligate (100%) HP:0001890
2 autoimmune thrombocytopenia 59 32 obligate (100%) Obligate (100%) HP:0001973
3 petechiae 59 32 hallmark (90%) Very frequent (99-80%) HP:0000967
4 autoimmune neutropenia 59 32 hallmark (90%) Very frequent (99-80%) HP:0001904
5 epistaxis 59 32 frequent (33%) Frequent (79-30%) HP:0000421
6 jaundice 59 32 frequent (33%) Frequent (79-30%) HP:0000952
7 bruising susceptibility 59 32 frequent (33%) Frequent (79-30%) HP:0000978
8 pallor 59 32 frequent (33%) Frequent (79-30%) HP:0000980
9 lethargy 59 32 frequent (33%) Frequent (79-30%) HP:0001254
10 muscle weakness 59 32 frequent (33%) Frequent (79-30%) HP:0001324
11 dyspnea 59 32 frequent (33%) Frequent (79-30%) HP:0002094
12 fatigue 59 32 frequent (33%) Frequent (79-30%) HP:0012378
13 syncope 59 32 occasional (7.5%) Occasional (29-5%) HP:0001279

GenomeRNAi Phenotypes related to Evans' Syndrome according to GeneCards Suite gene sharing:

26 (show all 24)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-105 9.77 TGFB1
2 Increased shRNA abundance (Z-score > 2) GR00366-A-116 9.77 TGFB1 AANAT
3 Increased shRNA abundance (Z-score > 2) GR00366-A-120 9.77 ADAMTS13 TGFB1
4 Increased shRNA abundance (Z-score > 2) GR00366-A-123 9.77 ADAMTS13
5 Increased shRNA abundance (Z-score > 2) GR00366-A-13 9.77 ADAMTS13
6 Increased shRNA abundance (Z-score > 2) GR00366-A-138 9.77 ADAMTS13
7 Increased shRNA abundance (Z-score > 2) GR00366-A-140 9.77 TGFB1
8 Increased shRNA abundance (Z-score > 2) GR00366-A-143 9.77 TGFB1
9 Increased shRNA abundance (Z-score > 2) GR00366-A-146 9.77 AANAT
10 Increased shRNA abundance (Z-score > 2) GR00366-A-151 9.77 ADAMTS13
11 Increased shRNA abundance (Z-score > 2) GR00366-A-157 9.77 AANAT
12 Increased shRNA abundance (Z-score > 2) GR00366-A-166 9.77 AANAT
13 Increased shRNA abundance (Z-score > 2) GR00366-A-169 9.77 TGFB1
14 Increased shRNA abundance (Z-score > 2) GR00366-A-178 9.77 ADAMTS13 TGFB1 AANAT
15 Increased shRNA abundance (Z-score > 2) GR00366-A-194 9.77 AANAT
16 Increased shRNA abundance (Z-score > 2) GR00366-A-196 9.77 AANAT
17 Increased shRNA abundance (Z-score > 2) GR00366-A-198 9.77 TGFB1
18 Increased shRNA abundance (Z-score > 2) GR00366-A-202 9.77 AANAT
19 Increased shRNA abundance (Z-score > 2) GR00366-A-205 9.77 AANAT
20 Increased shRNA abundance (Z-score > 2) GR00366-A-31 9.77 AANAT
21 Increased shRNA abundance (Z-score > 2) GR00366-A-35 9.77 TGFB1
22 Increased shRNA abundance (Z-score > 2) GR00366-A-36 9.77 ADAMTS13
23 Increased shRNA abundance (Z-score > 2) GR00366-A-56 9.77 TGFB1
24 Increased shRNA abundance (Z-score > 2) GR00366-A-97 9.77 ADAMTS13

MGI Mouse Phenotypes related to Evans' Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 endocrine/exocrine gland MP:0005379 9.55 RHBDF2 TGFB1 TPP2 AANAT CD40LG
2 hematopoietic system MP:0005397 9.43 TGFB1 TPP2 ADAMTS13 CD40LG HP RHBDF2
3 immune system MP:0005387 9.1 TGFB1 TPP2 ADAMTS13 CD40LG HP RHBDF2

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 5284616 6436030 46835353
4 Anti-Bacterial Agents Phase 1, Phase 2
5 Antibiotics, Antitubercular Phase 1, Phase 2
6 Immunosuppressive Agents Phase 1, Phase 2
7 Anti-Infective Agents Phase 1, Phase 2
8 Antifungal Agents 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
3 Severe Immune Cytopenia Registry Www.Sic-reg.Org Recruiting NCT03576742
4 EBMT ADWP Prospective Non-interventional Study: Post-AHSCT Management in SSC Patients (NISSC-2) Not yet recruiting NCT03444805

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:

41
T Cells, Thyroid, Bone, Bone Marrow, Pituitary, B Cells, Pancreas

Publications for Evans' Syndrome

Articles related to Evans' Syndrome:

(show top 50) (show all 71)
# Title Authors Year
1
Steroid-resistant autoimmune myelofibrosis in a patient with autoimmune hepatitis and Evans syndrome complicated with increased expression of TGF-I^ in the bone marrow: a case report. ( 28584963 )
2017
2
Persistent Fever in Medical Intensive Care Unit: Evans Syndrome Versus Actinomycotic Osteomyelitis? ( 28092284 )
2017
3
Association of autoimmune hepatitis type 1 in a child with Evans syndrome. ( 28878866 )
2017
4
Evans Syndrome Secondary to Common Variable Immune Deficiency. ( 26950085 )
2016
5
Evans syndrome after autologous peripheral blood stem cell transplantation for recurrent Hodgkin lymphoma. ( 27577012 )
2016
6
Evans syndrome with non-ST segment elevation myocardial infarction complicated by hemopericardium. ( 27751316 )
2016
7
Unusual co-occurrence of Evans syndrome and pure red cell aplasia in elderly patient with prostate cancer. ( 27465154 )
2016
8
Early-onset Evans syndrome, immunodeficiency, and premature immunosenescence associated with tripeptidyl-peptidase II deficiency. ( 25414442 )
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
Successful treatment with rituximab and romiplostim for thrombocytopenia associated with WaldenstrAPm's macroglobulinemia initially presenting as Evans syndrome. ( 25876786 )
2015
11
A Case of a TSH-secreting Pituitary Adenoma Associated with Evans' Syndrome. ( 26150183 )
2015
12
Profound autoimmune hemolysis and Evans syndrome in two asplenic patients with babesiosis. ( 25354478 )
2014
13
A case of Evans' syndrome following influenza vaccine. ( 22796025 )
2013
14
Hashimoto's thyroiditis associated Evans syndrome: A rare case report on the clustered autoimmune disease triad. ( 23961497 )
2013
15
Evaluation of children with chronic immune thrombocytopenic purpura and Evans syndrome treated with rituximab. ( 22815318 )
2013
16
CD56+ angioimmunoblastic T-cell lymphoma with evans syndrome : a case report and review of the literature. ( 23801132 )
2013
17
Refractory thrombocytopenia responds to octreotide treatment in a case of evans syndrome with gastric neuroendocrine tumor. ( 23984123 )
2013
18
Autoimmune hepatitis-primary biliary cirrhosis overlap syndrome concomitant with immune hemolytic anemia and immune thrombocytopenic purpura (Evans syndrome). ( 23273499 )
2013
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
Evans syndrome with cytomegalovirus infection followed by emerging peripheral T-cell lymphoma. ( 21437588 )
2012
22
Successful remission of Evans syndrome associated with Graves' disease by using propylthiouracil monotherapy. ( 21422690 )
2011
23
Successful rituximab therapy in refractory autoimmune hepatitis and Evans syndrome. ( 22446656 )
2011
24
Rituximab resistant evans syndrome and autoimmunity in Schimke immuno-osseous dysplasia. ( 21914180 )
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 control of steroid-intolerant Evans' syndrome associated with allogeneic peripheral blood hematopoietic stem cell transplant by rituximab. ( 21275632 )
2011
28
Glucocorticoid-resistant Evans' syndrome successfully controlled with low-dose cyclosporine. ( 21961487 )
2011
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
Identifying autoimmune lymphoproliferative syndrome in children with Evans syndrome: a multi-institutional study. ( 20068224 )
2010
32
Visceral leishmaniasis in a patient with common variable immunodeficiency and evans syndrome: clinical remarks. ( 19579705 )
2009
33
Amyloidosis, Evans syndrome and management options of lymphoplasmacytic lymphoma. ( 19291514 )
2009
34
Primary cutaneous nocardiosis in a patient with Evans' Syndrome. ( 19661017 )
2009
35
Complications of Evans' syndrome in an infant with hereditary spherocytosis: a case report. ( 19740448 )
2009
36
Splenic marginal zone lymphoma with Evans' syndrome, autoimmunity, and peripheral gamma/delta T cells. ( 18665362 )
2009
37
Treatment of Evans' syndrome with human intravenous immunoglobulin and leflunomide in a diabetic dog. ( 19411652 )
2009
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
Pseudohypoparathyroidism Ia with Evans syndrome. ( 18799944 )
2008
41
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
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
Increased lymphocyte Fas expression and high incidence of common variable immunodeficiency disorder in childhood Evans' syndrome. ( 17936685 )
2007
45
Tubercular adenitis and Evans' syndrome. ( 16539285 )
2006
46
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
47
A 20-year-old woman with Hashimoto's thyroiditis and Evans' syndrome. ( 16807996 )
2006
48
Pegylated interferon alpha-2a-associated life-threatening Evans' syndrome in a patient with chronic hepatitis C. ( 16230187 )
2005
49
Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS). ( 15542578 )
2005
50
Successful treatment of a patient with mixed warm and cold antibody mediated Evans syndrome and glucose intolerance. ( 15704210 )
2005

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 10.31 CD40LG TGFB1

GO Terms for Evans' Syndrome

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

# Name GO ID Score Top Affiliating Genes
1 protein processing GO:0016485 8.96 ADAMTS13 RHBDF2
2 proteolysis GO:0006508 8.92 ADAMTS13 HP RHBDF2 TPP2

Molecular functions related to Evans' Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 serine-type endopeptidase activity GO:0004252 8.8 HP RHBDF2 TPP2

Sources for Evans' Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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