MCID: EVN001
MIFTS: 50

Evans' Syndrome malady

Immune, Blood categories

Summaries for Evans' Syndrome

Sources:
43NIH Rare Diseases, 64Wikipedia, 33MalaCards
See all sources

Fully expand this MalaCard

Export this MalaCard
NIH Rare Diseases:43 Evans syndrome is a very rare autoimmune disorder in which the body makes antibodies that destroy the red blood cells, platelets and white blood cells. affected individuals usually experience thrombocytopenia (too few platelets) and coombs' positive hemolytic anemia (premature destruction of red blood cells).  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 vasculitis and autoimmune hemolytic anemia. An important gene associated with Evans' Syndrome is ADAMTS13 (ADAM metallopeptidase with thrombospondin type 1 motif, 13), and among its related pathways is amb2 Integrin signaling. The compounds warfarin and ticlopidine have been mentioned in the context of this disorder. Affiliated tissues include bone marrow, lung and pancreas, and related mouse phenotypes are immune system and hematopoietic system.

Wikipedia:64 Evans syndrome is an autoimmune disease in which an individual\'s antibodies attack their own red blood... more...

Aliases & Classifications for Evans' Syndrome

Sources:
8Disease Ontology, 61UMLS, 43NIH Rare Diseases, 45Novoseek, 49Orphanet, 27ICD9CM, 57SNOMED-CT, 40NCIt, 35MeSH, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 25ICD10
See all sources

Classifications:

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


Characteristics (Orphanet epidemiological data):

49
evans syndrome:
Inheritance: Sporadic; Prevalence: 1-9/1000000; Age of onset: Variable


Aliases & Descriptions:

evans' syndrome 8
evans syndrome 8 43 45 49
other primary thrombocytopenia 8 61
primary thrombocytopenia 8 61
evan's syndrome 8 61
autoimmune hemolytic anemia and autoimmune thrombocytopenia 43
primary thrombocytopenia nos 8
thrombocytopenia: primary 8
idiopathic disorder 45
idopathic purpuric 8
evan syndrome 43
idiopathic 8
purpuric 8


External Ids:

Disease Ontology8 DOID:8931, DOID:8925
ICD9CM27 287.32, 287.39
NCIt40 C61284
MeSH35 C536380
ICD10 via Orphanet26 D69.3
SNOMED-CT via Orphanet58 75331009
ICD1025 D69.4

Related Diseases for Evans' Syndrome

Sources:
17GeneCards, 18GeneDecks
See all sources

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

(show top 50)    (show all 1463)
idRelated DiseaseScoreTop Affiliating Genes
1vasculitis30.7F2, APOH
2autoimmune hemolytic anemia30.5HP
3hemochromatosis30.4HP
4nephritis30.4APOH
5venous thrombosis30.3F2, APOH
6systemic lupus erythematosus30.3F2, ADAMTS13, IL6, APOH
7pregnancy loss30.3APOH, F2
8acute pancreatitis30.2IL6
9portal vein thrombosis30.2F2
10thromboembolism30.2APOH, F2
11autoimmune thrombocytopenic purpura30.2PSMG3
12thrombophilia30.2APOH, F2
13cryoglobulinemia30.1APOH
14pulmonary disease, chronic obstructive30.1IL6
15thrombotic thrombocytopenic purpura, acquired30.1APOH, HP, ADAMTS13
16atherosclerosis30.0HP, IL6, APOH
17deficiency anemia30.0ADAMTS13, HP, F2, IL6
18vascular disease30.0IL6, HP, APOH
19antiphospholipid syndrome30.0APOH, F2, ADAMTS13
20stroke, ischemic30.0APOH
21hepatitis c29.9F2, HP
22thrombophlebitis29.9APOH
23hyperhomocysteinemia29.9APOH, F2
24thrombocytopenia29.9APOH, HP, IL6, ADAMTS13, F2
25thrombocytosis29.9IL6, HP, F2
26mixed connective tissue disease29.8APOH
27hemolytic-uremic syndrome29.7ADAMTS13
28central retinal vein occlusion29.7APOH
29protein c deficiency29.7APOH, F2
30acute myocardial infarction29.6ADAMTS13
31coronary heart disease29.6ADAMTS13, IL6
32coronary artery disease,29.6IL6, HP, ADAMTS13
33sepsis29.6IL6, ADAMTS13, F2
34acquired immunodeficiency syndrome29.4IL6
35sneddon syndrome29.4APOH
36syphilis29.4APOH
37viral hepatitis29.4F2
38hellp syndrome29.4HP, APOH, ADAMTS13
39idiopathic pulmonary fibrosis11.1
40arthritis11.0
41diffuse idiopathic skeletal hyperostosis10.8
42short stature10.8
43normal pressure hydrocephalus10.8
44hypereosinophilic syndrome10.7
45retroperitoneal fibrosis10.7
46hemosiderosis10.7
47idiopathic adolescent scoliosis10.7
48clubfoot10.6
49idiopathic edema10.6
50urticaria10.6

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



Diseases related to evans' syndrome

Clinical Features for Evans' Syndrome

Drugs & Therapeutics for Evans' Syndrome

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Evans' Syndrome

Drug clinical trials:

Search ClinicalTrials for Evans' Syndrome

Search NIH Clinical Center for Evans' Syndrome

Search CenterWatch for Evans' Syndrome

Genetic Tests for Evans' Syndrome

Anatomical Context for Evans' Syndrome

Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Evans' Syndrome:

33
Bone marrow, Lung, Pancreas, Thyroid, T cells

Animal Models for Evans' Syndrome or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Evans' Syndrome:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053877.7ADAMTS13, HP, IL6, F2, SMARCAL1
2MP:00053977.4ADAMTS13, HP, IL6, F2, SMARCAL1
3MP:00053767.3APOH, HP, IL6, F2, SMARCAL1, ADAMTS13
4MP:00107687.2ADAMTS13, SMARCAL1, F2, IL6, HP, APOH

Publications for Evans' Syndrome

Sources:
51PubMed
See all sources

Articles related to Evans' Syndrome:

(show all 44)
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
Mechanism of hepatorenal syndrome in rats of Long-Evans Cinnamon strain, an animal model of fulminant Wilson's disease. (10433383)
1999
38
Overproduction of antiplatelet antibody against glycoprotein IIb after splenectomy in a patient with Evans syndrome resulting in acquired thrombasthenia. (9554455)
1998
39
Hereditary spastic paraplegia and Evans's syndrome. (8819560)
1996
40
Chronic inflammatory demyelinating polyradiculoneuropathy associated with Evans' syndrome. (1341469)
1992
41
Evans' syndrome in Castleman's disease: unusual presentation with rectal bleeding from angiodysplasia of cecum. (1468898)
1992
42
Evans' syndrome as a presenting manifestation of atypical paroxysmal cold hemoglobinuria. (3578344)
1987
43
Evans' syndrome associated with microcystic adenoma of the pancreas. (3815308)
1987
44
Superior sagittal sinus thrombosis associated with Evans' syndrome of haemolytic anaemia. (4056835)
1985

Genetic Variations for Evans' Syndrome

Expression for genes affiliated with Evans' Syndrome

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
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

Sources:
38NCBI BioSystems Database
See all sources

Pathways related to Evans' Syndrome according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.2IL6, HP

Compounds for genes affiliated with Evans' Syndrome

Sources:
45Novoseek, 50PharmGKB, 11DrugBank, 24HMDB, 60Tocris Bioscience, 29IUPHAR
See all sources

Compounds related to Evans' Syndrome according to GeneCards/GeneDecks:

(show all 34)
idCompoundScoreTop Affiliating Genes
1warfarin45 50 11 2413.0APOH
2ticlopidine45 1110.9ADAMTS13, APOH
3ethanolamine oleate45 1110.8F2, HP
4ristocetin459.8ADAMTS13, F2
5kaolin459.8F2, APOH
6coumarins459.7APOH, F2
711-dehydrothromboxane b2459.7APOH, F2
8desmopressin45 60 29 1112.7ADAMTS13, F2
9ulinastatin459.6HP, IL6
10phosphatidylethanolamine45 1110.5APOH, F2
11anaphylatoxin459.5IL6, HP
12lipoteichoic acid459.4APOH, IL6
13kininogen459.4APOH, HP, F2
14homocysteine45 2410.3APOH, HP, F2
15etanercept45 50 1111.3APOH, IL6
16aspirin45 50 29 2412.3F2, HP, APOH
17infliximab45 50 1111.2APOH, IL6
188-isoprostane459.2APOH, HP, IL6
19phospholipid459.2F2, HP, APOH
20dextran sulfate459.1IL6, APOH
21protamine459.1F2, IL6
22heparin45 29 11 2412.0ADAMTS13, F2, HP, APOH
23pge1459.0F2, IL6, APOH
24hyaluronic acid45 2410.0F2, IL6, HP
25ivig458.9IL6, APOH
26epinephrine45 11 2410.9HP, IL6, F2
27methotrexate45 50 1110.9APOH, IL6, F2
28rituximab45 50 1110.8ADAMTS13, IL6, HP, APOH
29endotoxin458.8ADAMTS13, IL6, HP, APOH
30cyclosporin a45 29 6010.6HP, IL6, F2, ADAMTS13
31lactate458.5APOH, HP, IL6, F2
32estrogen458.4APOH, HP, IL6, F2
33fibrinogen458.2APOH, HP, IL6, F2, ADAMTS13
34creatinine458.2APOH, HP, IL6, F2, ADAMTS13

GO Terms for genes affiliated with Evans' Syndrome

Sources:
16Gene Ontology
See all sources

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

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:0056157.8ADAMTS13, F2, IL6, HP, APOH

Biological processes related to Evans' Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1negative regulation of fibrinolysisGO:0519189.4F2, APOH
2positive regulation of blood coagulationGO:0301949.3APOH, F2
3blood coagulation, intrinsic pathwayGO:0075979.1F2, APOH
4acute-phase responseGO:0069538.7F2, IL6, HP
5platelet activationGO:0301688.7ADAMTS13, F2, IL6

Products for genes affiliated with Evans' Syndrome

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Evans' Syndrome

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet