MCID: EVN001
MIFTS: 46

Evans' Syndrome malady

Rare diseases, Blood diseases, Immune diseases categories
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Summaries for Evans' Syndrome

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NIH Rare Diseases:42 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 usually experience thrombocytopenia (too few platelets) and coombs' positive hemolytic anemia (premature destruction of red blood cells). signs and symptoms usually develop in childhood and may include purpura, paleness, fatigue, and light-headedness.the exact cause of this condition is unknown. last updated: 9/21/2011

MalaCards based summary: 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 marrow.

Wikipedia:65 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:
8Disease Ontology, 42NIH Rare Diseases, 44Novoseek, 48Orphanet, 62UMLS, 57SNOMED-CT, 27ICD9CM, 34MeSH, 39NCIt, 26ICD10 via Orphanet, 63UMLS via Orphanet
See all sources

Evans' Syndrome, Aliases & Descriptions:

Name: Evans' Syndrome 8
Evans Syndrome 8 42 44 48
Autoimmune Hemolytic Anemia and Autoimmune Thrombocytopenia 42 48
Primary Thrombocytopenia 8 62
Idiopathic Disorder 44 62
Evan's Syndrome 8 62
 
Thrombocytopenia: Primary 8
Immune Pancytopenia 48
Idopathic Purpuric 8
Evan Syndrome 42
Idiopathic 8
Purpuric 8


Classifications:

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


Characteristics (Orphanet epidemiological data):

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


External Ids:

Disease Ontology8 DOID:8931, DOID:8925
ICD9CM27 287.32, 287.39
MeSH34 C536380
NCIt39 C61284
ICD10 via Orphanet26 D69.3
UMLS via Orphanet63 C0272126

Related Diseases for Evans' Syndrome

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

(show top 50)    (show all 1757)
idRelated DiseaseScoreTop Affiliating Genes
1hemolytic anemia30.5ADAMTS13, HP
2proteinuria30.2ADAMTS13, HP
3liver cirrhosis30.0HP, ADAMTS13
4thrombocytopenia30.0HP, ADAMTS13
5deficiency anemia29.9HP, ADAMTS13
6thrombotic thrombocytopenic purpura, acquired29.8ADAMTS13, HP
7primary hyperoxaluria29.7HP, ADAMTS13
8ischemia29.6ADAMTS13, HP
9hellp syndrome29.5HP, ADAMTS13
10idiopathic pulmonary fibrosis11.1
11pulmonary fibrosis11.1
12hypertension11.0
13arthritis11.0
14intracranial hypertension10.9
15diffuse idiopathic skeletal hyperostosis10.9
16hyperostosis10.9
17idiopathic generalized epilepsy10.9
18short stature10.8
19hydrocephalus10.8
20normal pressure hydrocephalus10.8
21dilated cardiomyopathy10.8
22nephrotic syndrome10.8
23hypereosinophilic syndrome10.8
24retroperitoneal fibrosis10.7
25hemosiderosis10.7
26retinitis10.7
27glomerulonephritis10.7
28infertility10.7
29adolescent idiopathic scoliosis10.7
30dystonia10.7
31clubfoot10.7
32idiopathic interstitial pneumonia10.7
33macular holes10.7
34urticaria10.6
35systemic onset juvenile idiopathic arthritis10.6
36t-lymphocytopenia10.6
37hypoparathyroidism10.6
38myopathy10.6
39pancreatitis10.6
40myelofibrosis10.6
41sensorineural hearing loss10.6
42hypogonadism10.6
43idiopathic edema10.6
44uveitis10.6
45parkinson's disease10.6
46cervicitis10.6
47osteoporosis10.6
48intestinal pseudo-obstruction10.6
49male infertility10.6
50membranous glomerulonephritis10.6

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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Drug clinical trials:

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Genetic Tests for Evans' Syndrome

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Anatomical Context for Evans' Syndrome

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MalaCards organs/tissues related to Evans' Syndrome:

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

Animal Models for Evans' Syndrome or affiliated genes

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Publications for Evans' Syndrome

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Articles related to Evans' Syndrome:

(show all 44)
idTitleAuthorsYear
1
Profound autoimmune hemolysis and Evans syndrome in two asplenic patients with babesiosis. (25354478)
2014
2
CD56+ angioimmunoblastic T-cell lymphoma with evans syndrome : a case report and review of the literature. (23801132)
2013
3
Evaluation of children with chronic immune thrombocytopenic purpura and Evans syndrome treated with rituximab. (22815318)
2013
4
Refractory thrombocytopenia responds to octreotide treatment in a case of evans syndrome with gastric neuroendocrine tumor. (23984123)
2013
5
Autoimmune hepatitis-primary biliary cirrhosis overlap syndrome concomitant with immune hemolytic anemia and immune thrombocytopenic purpura (Evans syndrome). (23273499)
2013
6
Hashimoto's thyroiditis associated Evans syndrome: A rare case report on the clustered autoimmune disease triad. (23961497)
2013
7
Dermatomyositis associated with generalized subcutaneous edema and Evans syndrome. (22030016)
2012
8
Successful allogeneic cord blood transplantation in a patient with Evans syndrome leads to correction of hereditary angioedema type I as secondary effect. (22327128)
2012
9
Evans syndrome with cytomegalovirus infection followed by emerging peripheral T-cell lymphoma. (21437588)
2012
10
Evans syndrome following long-standing Hashimoto's thyroiditis and successful treatment with rituximab. (22259635)
2011
11
Successful remission of Evans syndrome associated with Graves' disease by using propylthiouracil monotherapy. (21422690)
2011
12
Successful rituximab therapy in refractory autoimmune hepatitis and Evans syndrome. (22446656)
2011
13
Rituximab resistant evans syndrome and autoimmunity in Schimke immuno-osseous dysplasia. (21914180)
2011
14
Massive hemoperitoneum with splenic infarction in Evans syndrome. (21088621)
2011
15
Howel-Evans syndrome: a variant of ectodermal dysplasia. (20486456)
2010
16
Identifying autoimmune lymphoproliferative syndrome in children with Evans syndrome: a multi-institutional study. (20068224)
2010
17
Complications of Evans' syndrome in an infant with hereditary spherocytosis: a case report. (19740448)
2009
18
Splenic marginal zone lymphoma with Evans' syndrome, autoimmunity, and peripheral gamma/delta T cells. (18665362)
2009
19
Primary cutaneous nocardiosis in a patient with Evans' Syndrome. (19661017)
2009
20
Visceral leishmaniasis in a patient with common variable immunodeficiency and evans syndrome: clinical remarks. (19579705)
2009
21
Amyloidosis, Evans syndrome and management options of lymphoplasmacytic lymphoma. (19291514)
2009
22
Pseudohypoparathyroidism Ia with Evans syndrome. (18799944)
2008
23
Consolidation treatment with rituximab induces complete and persistent remission of mixed type Evans syndrome. (18469554)
2008
24
Common variable immunodeficiency and Evans syndrome complicated by autoimmune hemolysis due to anti-Jka auto-antibodies. (18452064)
2008
25
Increased lymphocyte Fas expression and high incidence of common variable immunodeficiency disorder in childhood Evans' syndrome. (17936685)
2007
26
Autoimmune hemolytic anemias, Evans' syndromes, and pure red cell aplasia in non-Hodgkin lymphomas. (17577777)
2007
27
A case of pure red cell aplasia complicated by Evans syndrome. (17694270)
2007
28
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
29
Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS). (15542578)
2005
30
Successful treatment of a patient with mixed warm and cold antibody mediated Evans syndrome and glucose intolerance. (15704210)
2005
31
Rituximab treatment for relapsed autoimmune hemolytic anemia in Evans syndrome. (14686491)
2003
32
Acute inflammatory demyelinating polyneuropathy concurrent with Evans syndrome. (12602738)
2003
33
Rituximab for immune cytopenia in adults: idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia, and Evans syndrome. (14601692)
2003
34
Lung carcinoma with congenital plantar keratoderma as a variant of Clarke-Howel-Evans syndrome. (12786874)
2003
35
Bronchiolitis obliterans organizing pneumonia associated with Evans syndrome. (11786723)
2001
36
Th1 and Th2 cytokines in a patient with Evans' syndrome and profound lymphopenia. (11054090)
2000
37
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
38
Disseminated nocardiosis as a complication of Evans' syndrome. (10460354)
1999
39
Overproduction of antiplatelet antibody against glycoprotein IIb after splenectomy in a patient with Evans syndrome resulting in acquired thrombasthenia. (9554455)
1998
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

Variations for Evans' Syndrome

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Expression for genes affiliated with Evans' Syndrome

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Expression patterns in normal tissues for genes affiliated with Evans' Syndrome

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Pathways for genes affiliated with Evans' Syndrome

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Compounds for genes affiliated with Evans' Syndrome

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

idCompoundScoreTop Affiliating Genes
1rituximab44 50 1111.3HP, ADAMTS13
2urea44 24 1111.3HP, ADAMTS13
3endotoxin449.3HP, ADAMTS13
4nacl449.2HP, ADAMTS13
5fibrinogen449.2ADAMTS13, HP
6cyclosporin a44 28 6111.1HP, ADAMTS13
7steroid449.1HP, ADAMTS13
8creatinine449.0HP, ADAMTS13
9heparin44 28 24 1111.8HP, ADAMTS13

GO Terms for genes affiliated with Evans' Syndrome

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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

Sources for Evans' Syndrome

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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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet