MCID: EVN001
MIFTS: 46

Evans' Syndrome malady

Rare diseases, Immune diseases, Blood diseases categories

Summaries for Evans' Syndrome

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Sources:
44NIH Rare Diseases, 66Wikipedia, 34MalaCards
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NIH Rare Diseases:44 Evans syndrome is a very rareĀ autoimmune disorderĀ in which the body makes antibodies that destroy the red blood cells, platelets and white blood cells. affected individuals usually experienceĀ thrombocytopenia (too few platelets) and coombs' positive hemolytic anemia (premature destruction of red blood cells).Ā Ā people with evans syndrome may have low levels of all three types of blood cells at one time, or may only have problems with one or two of them. the exact cause of this condition is unknown. last updated: 9/21/2011

MalaCards: Evans' Syndrome, also known as evans syndrome, is related to hemolytic anemia and proteinuria. An important gene associated with Evans' Syndrome is ADAMTS13 (ADAM metallopeptidase with thrombospondin type 1 motif, 13). The compounds rituximab and urea have been mentioned in the context of this disorder. Affiliated tissues include t cells, thyroid and bone.

Wikipedia:66 Evans syndrome is an autoimmune disease in which an individual\'s antibodies attack their own red blood... more...

Aliases & Classifications for Evans' Syndrome

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Sources:
9Disease Ontology, 63UMLS, 44NIH Rare Diseases, 46Novoseek, 50Orphanet, 28ICD9CM, 36MeSH, 59SNOMED-CT, 41NCIt, 27ICD10 via Orphanet, 60SNOMED-CT via Orphanet, 64UMLS via Orphanet, 26ICD10
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Immune diseases, Blood diseases


Characteristics (Orphanet epidemiological data):

50
evans syndrome:
Inheritance: Sporadic; Prevalence: 1-9/1000000; Age of onset: Variable


Aliases & Descriptions:

evans' syndrome 9
evans syndrome 9 44 46 50
autoimmune hemolytic anemia and autoimmune thrombocytopenia 44 50
other primary thrombocytopenia 9 63
primary thrombocytopenia 9 63
evan's syndrome 9 63
primary thrombocytopenia nos 9
thrombocytopenia: primary 9
immune pancytopenia 50
idiopathic disorder 46
idopathic purpuric 9
evan syndrome 44
idiopathic 9
purpuric 9


External Ids:

Disease Ontology9 DOID:8931, DOID:8925
ICD9CM28 287.32, 287.39
MeSH36 C536380
NCIt41 C61284
ICD10 via Orphanet27 D69.3
SNOMED-CT via Orphanet60 75331009
UMLS via Orphanet64 C0272126
ICD1026 D69.4

Related Diseases for Evans' Syndrome

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Sources:
18GeneCards, 19GeneDecks
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Diseases related to Evans' Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 1740)
idRelated DiseaseScoreTop Affiliating Genes
1hemolytic anemia30.5ADAMTS13, HP
2proteinuria30.3ADAMTS13, HP
3liver cirrhosis30.0HP, ADAMTS13
4thrombocytopenia30.0ADAMTS13, HP
5deficiency anemia30.0ADAMTS13, HP
6primary hyperoxaluria29.8ADAMTS13, HP
7thrombotic thrombocytopenic purpura, acquired29.7HP, ADAMTS13
8coronary artery disease29.6ADAMTS13, HP
9ischemia29.6HP, ADAMTS13
10hellp syndrome29.4ADAMTS13, HP
11idiopathic pulmonary fibrosis11.1
12pulmonary fibrosis11.1
13hypertension11.0
14arthritis11.0
15intracranial hypertension10.9
16diffuse idiopathic skeletal hyperostosis10.8
17hyperostosis10.8
18idiopathic generalized epilepsy10.8
19short stature10.8
20hydrocephalus10.8
21normal pressure hydrocephalus10.8
22dilated cardiomyopathy10.8
23nephrotic syndrome10.7
24hypereosinophilic syndrome10.7
25hemosiderosis10.7
26retroperitoneal fibrosis10.7
27glomerulonephritis10.7
28retinitis10.7
29infertility10.7
30dystonia10.7
31clubfoot10.6
32idiopathic interstitial pneumonia10.6
33macular holes10.6
34urticaria10.6
35systemic onset juvenile idiopathic arthritis10.6
36t-lymphocytopenia10.6
37hypoparathyroidism10.6
38pancreatitis10.6
39myelofibrosis10.6
40myopathy10.6
41hypogonadism10.6
42idiopathic edema10.6
43uveitis10.6
44sensorineural hearing loss10.6
45parkinson's disease10.6
46cervicitis10.6
47osteoporosis10.5
48membranous glomerulonephritis10.5
49intestinal pseudo-obstruction10.5
50hypogonadotropism10.5

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



Diseases related to evans' syndrome

Symptoms for Evans' Syndrome

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Drugs & Therapeutics for Evans' Syndrome

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
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Approved drugs:

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Drug clinical trials:

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Search CenterWatch for Evans' Syndrome

Genetic Tests for Evans' Syndrome

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Anatomical Context for Evans' Syndrome

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Sources:
34MalaCards
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MalaCards organs/tissues related to Evans' Syndrome:

34
T cells, Thyroid, Bone, Lung, Bone marrow, Pancreas

Animal Models for Evans' Syndrome or affiliated genes

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Publications for Evans' Syndrome

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Sources:
53PubMed
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Articles related to Evans' Syndrome:

(show all 42)
idTitleAuthorsYear
1
CD56+ angioimmunoblastic T-cell lymphoma with evans syndrome : a case report and review of the literature. (23801132)
2013
2
Evaluation of children with chronic immune thrombocytopenic purpura and Evans syndrome treated with rituximab. (22815318)
2013
3
Refractory thrombocytopenia responds to octreotide treatment in a case of evans syndrome with gastric neuroendocrine tumor. (23984123)
2013
4
Autoimmune hepatitis-primary biliary cirrhosis overlap syndrome concomitant with immune hemolytic anemia and immune thrombocytopenic purpura (Evans syndrome). (23273499)
2013
5
Hashimoto's thyroiditis associated Evans syndrome: A rare case report on the clustered autoimmune disease triad. (23961497)
2013
6
Dermatomyositis associated with generalized subcutaneous edema and Evans syndrome. (22030016)
2012
7
Successful allogeneic cord blood transplantation in a patient with Evans syndrome leads to correction of hereditary angioedema type I as secondary effect. (22327128)
2012
8
Evans syndrome with cytomegalovirus infection followed by emerging peripheral T-cell lymphoma. (21437588)
2012
9
Evans syndrome following long-standing Hashimoto's thyroiditis and successful treatment with rituximab. (22259635)
2011
10
Successful remission of Evans syndrome associated with Graves' disease by using propylthiouracil monotherapy. (21422690)
2011
11
Successful rituximab therapy in refractory autoimmune hepatitis and Evans syndrome. (22446656)
2011
12
Massive hemoperitoneum with splenic infarction in Evans syndrome. (21088621)
2011
13
Howel-Evans syndrome: a variant of ectodermal dysplasia. (20486456)
2010
14
Identifying autoimmune lymphoproliferative syndrome in children with Evans syndrome: a multi-institutional study. (20068224)
2010
15
Complications of Evans' syndrome in an infant with hereditary spherocytosis: a case report. (19740448)
2009
16
Splenic marginal zone lymphoma with Evans' syndrome, autoimmunity, and peripheral gamma/delta T cells. (18665362)
2009
17
Primary cutaneous nocardiosis in a patient with Evans' Syndrome. (19661017)
2009
18
Visceral leishmaniasis in a patient with common variable immunodeficiency and evans syndrome: clinical remarks. (19579705)
2009
19
Amyloidosis, Evans syndrome and management options of lymphoplasmacytic lymphoma. (19291514)
2009
20
Pseudohypoparathyroidism Ia with Evans syndrome. (18799944)
2008
21
Consolidation treatment with rituximab induces complete and persistent remission of mixed type Evans syndrome. (18469554)
2008
22
Common variable immunodeficiency and Evans syndrome complicated by autoimmune hemolysis due to anti-Jka auto-antibodies. (18452064)
2008
23
Increased lymphocyte Fas expression and high incidence of common variable immunodeficiency disorder in childhood Evans' syndrome. (17936685)
2007
24
Autoimmune hemolytic anemias, Evans' syndromes, and pure red cell aplasia in non-Hodgkin lymphomas. (17577777)
2007
25
A case of pure red cell aplasia complicated by Evans syndrome. (17694270)
2007
26
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
27
Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS). (15542578)
2005
28
Successful treatment of a patient with mixed warm and cold antibody mediated Evans syndrome and glucose intolerance. (15704210)
2005
29
Rituximab treatment for relapsed autoimmune hemolytic anemia in Evans syndrome. (14686491)
2003
30
Acute inflammatory demyelinating polyneuropathy concurrent with Evans syndrome. (12602738)
2003
31
Rituximab for immune cytopenia in adults: idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia, and Evans syndrome. (14601692)
2003
32
Lung carcinoma with congenital plantar keratoderma as a variant of Clarke-Howel-Evans syndrome. (12786874)
2003
33
Bronchiolitis obliterans organizing pneumonia associated with Evans syndrome. (11786723)
2001
34
Th1 and Th2 cytokines in a patient with Evans' syndrome and profound lymphopenia. (11054090)
2000
35
An immunological syndrome featuring transverse myelitis, Evans syndrome and pulmonary infiltrates after unrelated bone marrow transplant in a patient with severe aplastic anemia. (11149736)
2000
36
Disseminated nocardiosis as a complication of Evans' syndrome. (10460354)
1999
37
Overproduction of antiplatelet antibody against glycoprotein IIb after splenectomy in a patient with Evans syndrome resulting in acquired thrombasthenia. (9554455)
1998
38
Chronic inflammatory demyelinating polyradiculoneuropathy associated with Evans' syndrome. (1341469)
1992
39
Evans' syndrome in Castleman's disease: unusual presentation with rectal bleeding from angiodysplasia of cecum. (1468898)
1992
40
Evans' syndrome as a presenting manifestation of atypical paroxysmal cold hemoglobinuria. (3578344)
1987
41
Evans' syndrome associated with microcystic adenoma of the pancreas. (3815308)
1987
42
Superior sagittal sinus thrombosis associated with Evans' syndrome of haemolytic anaemia. (4056835)
1985

Variations for Evans' Syndrome

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Expression for genes affiliated with Evans' Syndrome

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Sources:
2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Evans' Syndrome

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

Pathways for genes affiliated with Evans' Syndrome

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Compounds for genes affiliated with Evans' Syndrome

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Sources:
46Novoseek, 52PharmGKB, 12DrugBank, 25HMDB, 30IUPHAR, 62Tocris Bioscience
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Compounds related to Evans' Syndrome according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1rituximab46 52 1211.3HP, ADAMTS13
2urea46 25 1211.3HP, ADAMTS13
3endotoxin469.3HP, ADAMTS13
4nacl469.2HP, ADAMTS13
5fibrinogen469.2ADAMTS13, HP
6cyclosporin a46 30 6211.1HP, ADAMTS13
7steroid469.1HP, ADAMTS13
8creatinine469.0HP, ADAMTS13
9heparin46 30 25 1211.8HP, ADAMTS13

GO Terms for genes affiliated with Evans' Syndrome

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17Gene Ontology
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Cellular components related to Evans' Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:0056159.1HP, ADAMTS13

Products for genes affiliated with Evans' Syndrome

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Evans' Syndrome

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet