MCID: EVN001
MIFTS: 54

Evans' Syndrome malady

Immune diseases, Blood diseases categories

Summaries for Evans' Syndrome

About this section
Sources:
42NIH Rare Diseases, 63Wikipedia, 32MalaCards
See all sources

Fully expand this MalaCard

Export this MalaCard
NIH Rare Diseases:42 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 hypertension and nephrotic syndrome. 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 t cells, thyroid and bone marrow, and related mouse phenotypes are immune system and hematopoietic system.

Wikipedia:63 Evans syndrome is an autoimmune disease in which an individual\'s antibodies attack their own red blood... more...

Aliases & Classifications for Evans' Syndrome

About this section
Sources:
8Disease Ontology, 42NIH Rare Diseases, 44Novoseek, 48Orphanet, 60UMLS, 27ICD9CM, 56SNOMED-CT, 39NCIt, 34MeSH, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 25ICD10
See all sources

Classifications:

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


Characteristics (Orphanet epidemiological data):

48
evans syndrome:
Inheritance: Sporadic; Prevalence: 1-9/1000000; Age of onset: Variable


Aliases & Descriptions:

evans' syndrome 8
evans syndrome 8 42 44 48
other primary thrombocytopenia 8 60
primary thrombocytopenia 8 60
evan's syndrome 8 60
autoimmune hemolytic anemia and autoimmune thrombocytopenia 42
primary thrombocytopenia nos 8
thrombocytopenia: primary 8
idiopathic disorder 44
idopathic purpuric 8
evan syndrome 42
idiopathic 8
purpuric 8


External Ids:

Disease Ontology8 DOID:8931, DOID:8925
ICD9CM27 287.32, 287.39
NCIt39 C61284
MeSH34 C536380
ICD10 via Orphanet26 D69.3
SNOMED-CT via Orphanet57 75331009
ICD1025 D69.4

Related Diseases for Evans' Syndrome

About this section
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 1536)
idRelated DiseaseScoreTop Affiliating Genes
1hypertension31.4F2
2nephrotic syndrome31.0SMARCAL1, HP
3vasculitis30.7F2, APOH
4autoimmune hemolytic anemia30.4HP
5hemolytic anemia30.4APOH, HP, ADAMTS13
6hemochromatosis30.4HP
7diabetes mellitus30.3IL6, HP
8lupus erythematosus30.3APOH, F2
9purpura30.3ADAMTS13
10systemic lupus erythematosus30.3ADAMTS13, F2, IL6, APOH
11connective tissue disease30.2APOH, ADAMTS13
12proteinuria30.2ADAMTS13, HP, APOH
13acute pancreatitis30.2IL6
14portal vein thrombosis30.2F2
15inflammatory bowel disease30.1IL6
16autoimmune thrombocytopenic purpura30.1PSMG3
17thrombophilia30.1F2, APOH
18cryoglobulinemia30.1APOH
19essential hypertension30.0HP
20atherosclerosis30.0APOH, HP, IL6
21deficiency anemia30.0HP, IL6, F2, ADAMTS13
22retinal artery occlusion30.0APOH
23migraine30.0APOH
24vascular disease30.0IL6, HP, APOH
25liver cirrhosis30.0HP, F2, ADAMTS13
26antiphospholipid syndrome30.0APOH, F2, ADAMTS13
27stroke, ischemic29.9APOH
28hepatitis c29.9F2, HP
29pulmonary embolism29.9F2, APOH
30pneumonia29.9IL6
31myocardial infarction29.9ADAMTS13
32thrombophlebitis29.9APOH
33thrombocytopenia29.9APOH, HP, IL6, F2, ADAMTS13
34hyperhomocysteinemia29.9F2, APOH
35thrombocytosis29.9HP, IL6, F2
36thrombotic thrombocytopenic purpura, congenital29.8ADAMTS13, HP
37mixed connective tissue disease29.8APOH
38multiple myeloma29.8IL6
39myeloma29.8IL6
40disseminated intravascular coagulation29.8F2, ADAMTS13
41hemolytic-uremic syndrome29.7ADAMTS13
42central retinal vein occlusion29.7APOH
43retinal vein occlusion29.7APOH
44cerebrovascular disease29.7APOH
45protein c deficiency29.7F2, APOH
46acute myocardial infarction29.6ADAMTS13
47skin disease29.6IL6
48ischemia29.6ADAMTS13, HP
49thrombotic thrombocytopenic purpura, acquired29.6APOH, HP, ADAMTS13
50coronary artery disease29.6ADAMTS13, IL6, HP

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



Diseases related to evans' syndrome

Clinical Features for Evans' Syndrome

About this section

Drugs & Therapeutics for Evans' Syndrome

About this section
Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-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

About this section

Anatomical Context for Evans' Syndrome

About this section
Sources:
32MalaCards
See all sources

MalaCards organs/tissues related to Evans' Syndrome:

32
T cells, Thyroid, Bone marrow, Bone, Lung, Pancreas

Animal Models for Evans' Syndrome or affiliated genes

About this section
Sources:
36MGI
See all sources

MGI Mouse Phenotypes related to Evans' Syndrome:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053877.7ADAMTS13, SMARCAL1, F2, IL6, HP
2MP:00053977.4HP, IL6, F2, SMARCAL1, ADAMTS13
3MP:00053767.3ADAMTS13, SMARCAL1, F2, IL6, HP, APOH
4MP:00107687.2APOH, HP, IL6, F2, SMARCAL1, ADAMTS13

Publications for Evans' Syndrome

About this section
Sources:
50PubMed
See all sources

Articles related to Evans' Syndrome:

(show all 43)
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
Chronic inflammatory demyelinating polyradiculoneuropathy associated with Evans' syndrome. (1341469)
1992
40
Evans' syndrome in Castleman's disease: unusual presentation with rectal bleeding from angiodysplasia of cecum. (1468898)
1992
41
Evans' syndrome as a presenting manifestation of atypical paroxysmal cold hemoglobinuria. (3578344)
1987
42
Evans' syndrome associated with microcystic adenoma of the pancreas. (3815308)
1987
43
Superior sagittal sinus thrombosis associated with Evans' syndrome of haemolytic anaemia. (4056835)
1985

Genetic Variations for Evans' Syndrome

About this section

Expression for genes affiliated with Evans' Syndrome

About this section
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

About this section
Sources:
37NCBI 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

About this section
Sources:
44Novoseek, 49PharmGKB, 11DrugBank, 24HMDB, 59Tocris Bioscience, 28IUPHAR
See all sources

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

(show all 34)
idCompoundScoreTop Affiliating Genes
1warfarin44 49 11 2413.0APOH
2ticlopidine44 1110.9ADAMTS13, APOH
3ethanolamine oleate44 1110.8F2, HP
4ristocetin449.8ADAMTS13, F2
5kaolin449.8F2, APOH
6coumarins449.7APOH, F2
711-dehydrothromboxane b2449.7APOH, F2
8desmopressin44 59 28 1112.7ADAMTS13, F2
9ulinastatin449.6HP, IL6
10phosphatidylethanolamine44 1110.5APOH, F2
11anaphylatoxin449.5IL6, HP
12lipoteichoic acid449.4APOH, IL6
13kininogen449.4APOH, HP, F2
14homocysteine44 2410.3APOH, HP, F2
15etanercept44 49 1111.3APOH, IL6
16aspirin44 49 28 2412.3F2, HP, APOH
17infliximab44 49 1111.2APOH, IL6
188-isoprostane449.2APOH, HP, IL6
19phospholipid449.2F2, HP, APOH
20dextran sulfate449.1IL6, APOH
21protamine449.1F2, IL6
22heparin44 28 11 2412.0ADAMTS13, F2, HP, APOH
23pge1449.0F2, IL6, APOH
24hyaluronic acid44 2410.0F2, IL6, HP
25ivig448.9IL6, APOH
26epinephrine44 11 2410.9HP, IL6, F2
27methotrexate44 49 1110.9APOH, IL6, F2
28rituximab44 49 1110.8ADAMTS13, IL6, HP, APOH
29endotoxin448.8ADAMTS13, IL6, HP, APOH
30cyclosporin a44 28 5910.6HP, IL6, F2, ADAMTS13
31lactate448.5APOH, HP, IL6, F2
32estrogen448.4APOH, HP, IL6, F2
33fibrinogen448.2APOH, HP, IL6, F2, ADAMTS13
34creatinine448.2APOH, HP, IL6, F2, ADAMTS13

GO Terms for genes affiliated with Evans' Syndrome

About this section
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

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Evans' Syndrome

About this section
3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet