MCID: EVN001
MIFTS: 39

Evans' Syndrome malady

Rare diseases, Blood diseases, Immune diseases categories

Aliases & Classifications for Evans' Syndrome

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Sources:
9Disease Ontology, 41NIH Rare Diseases, 43Novoseek, 47Orphanet, 60UMLS, 38NCIt, 27ICD9CM, 33MeSH, 55SNOMED-CT, 26ICD10 via Orphanet, 61UMLS via Orphanet
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Evans' Syndrome, Aliases & Descriptions:

Name: Evans' Syndrome 9
Evans Syndrome 9 41 43 47
Autoimmune Hemolytic Anemia and Autoimmune Thrombocytopenia 41 47
Primary Thrombocytopenia 9 60
Immune Pancytopenia 41 47
Evan's Syndrome 9 60
Thrombocytopenia: Primary 9
 
Idiopathic Disorder 43
Idiopathic Disease 60
Idopathic Purpuric 9
Evan Syndrome 41
Idiopathic 9
Purpuric 9


Classifications:

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


Characteristics (Orphanet epidemiological data):

47
evans syndrome:
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe); Age of onset: Adult,Childhood


External Ids:

Disease Ontology9 DOID:8931, DOID:8925
NCIt38 C61284
ICD9CM27 287.32, 287.39
MeSH33 C536380
Orphanet47 1959
ICD10 via Orphanet26 D69.3
UMLS via Orphanet61 C0272126

Summaries for Evans' Syndrome

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NIH Rare Diseases:41 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. last updated: 11/17/2014

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

Related Diseases for Evans' Syndrome

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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:

Search ClinicalTrials for Evans' Syndrome

Search NIH Clinical Center for Evans' Syndrome

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:

31
T cells, Thyroid, Bone marrow, Bone, Colon, 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 45)
idTitleAuthorsYear
1
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
2
Successful treatment with rituximab and romiplostim for thrombocytopenia associated with WaldenstrAPm's macroglobulinemia initially presenting as Evans syndrome. (25876786)
2015
3
Profound autoimmune hemolysis and Evans syndrome in two asplenic patients with babesiosis. (25354478)
2014
4
CD56+ angioimmunoblastic T-cell lymphoma with evans syndrome : a case report and review of the literature. (23801132)
2013
5
Evaluation of children with chronic immune thrombocytopenic purpura and Evans syndrome treated with rituximab. (22815318)
2013
6
Refractory thrombocytopenia responds to octreotide treatment in a case of evans syndrome with gastric neuroendocrine tumor. (23984123)
2013
7
Autoimmune hepatitis-primary biliary cirrhosis overlap syndrome concomitant with immune hemolytic anemia and immune thrombocytopenic purpura (Evans syndrome). (23273499)
2013
8
Hashimoto's thyroiditis associated Evans syndrome: A rare case report on the clustered autoimmune disease triad. (23961497)
2013
9
Dermatomyositis associated with generalized subcutaneous edema and Evans syndrome. (22030016)
2012
10
Successful allogeneic cord blood transplantation in a patient with Evans syndrome leads to correction of hereditary angioedema type I as secondary effect. (22327128)
2012
11
Evans syndrome with cytomegalovirus infection followed by emerging peripheral T-cell lymphoma. (21437588)
2012
12
Evans syndrome following long-standing Hashimoto's thyroiditis and successful treatment with rituximab. (22259635)
2011
13
Successful remission of Evans syndrome associated with Graves' disease by using propylthiouracil monotherapy. (21422690)
2011
14
Successful rituximab therapy in refractory autoimmune hepatitis and Evans syndrome. (22446656)
2011
15
Rituximab resistant evans syndrome and autoimmunity in Schimke immuno-osseous dysplasia. (21914180)
2011
16
Massive hemoperitoneum with splenic infarction in Evans syndrome. (21088621)
2011
17
Howel-Evans syndrome: a variant of ectodermal dysplasia. (20486456)
2010
18
Identifying autoimmune lymphoproliferative syndrome in children with Evans syndrome: a multi-institutional study. (20068224)
2010
19
Complications of Evans' syndrome in an infant with hereditary spherocytosis: a case report. (19740448)
2009
20
Splenic marginal zone lymphoma with Evans' syndrome, autoimmunity, and peripheral gamma/delta T cells. (18665362)
2009
21
Primary cutaneous nocardiosis in a patient with Evans' Syndrome. (19661017)
2009
22
Visceral leishmaniasis in a patient with common variable immunodeficiency and evans syndrome: clinical remarks. (19579705)
2009
23
Amyloidosis, Evans syndrome and management options of lymphoplasmacytic lymphoma. (19291514)
2009
24
Pseudohypoparathyroidism Ia with Evans syndrome. (18799944)
2008
25
Consolidation treatment with rituximab induces complete and persistent remission of mixed type Evans syndrome. (18469554)
2008
26
Common variable immunodeficiency and Evans syndrome complicated by autoimmune hemolysis due to anti-Jka auto-antibodies. (18452064)
2008
27
Increased lymphocyte Fas expression and high incidence of common variable immunodeficiency disorder in childhood Evans' syndrome. (17936685)
2007
28
Autoimmune hemolytic anemias, Evans' syndromes, and pure red cell aplasia in non-Hodgkin lymphomas. (17577777)
2007
29
A case of pure red cell aplasia complicated by Evans syndrome. (17694270)
2007
30
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
31
Unmasking Evans syndrome: T-cell phenotype and apoptotic response reveal autoimmune lymphoproliferative syndrome (ALPS). (15542578)
2005
32
Successful treatment of a patient with mixed warm and cold antibody mediated Evans syndrome and glucose intolerance. (15704210)
2005
33
Rituximab treatment for relapsed autoimmune hemolytic anemia in Evans syndrome. (14686491)
2003
34
Acute inflammatory demyelinating polyneuropathy concurrent with Evans syndrome. (12602738)
2003
35
Rituximab for immune cytopenia in adults: idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia, and Evans syndrome. (14601692)
2003
36
Lung carcinoma with congenital plantar keratoderma as a variant of Clarke-Howel-Evans syndrome. (12786874)
2003
37
Bronchiolitis obliterans organizing pneumonia associated with Evans syndrome. (11786723)
2001
38
Th1 and Th2 cytokines in a patient with Evans' syndrome and profound lymphopenia. (11054090)
2000
39
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
40
Disseminated nocardiosis as a complication of Evans' syndrome. (10460354)
1999
41
Chronic inflammatory demyelinating polyradiculoneuropathy associated with Evans' syndrome. (1341469)
1992
42
Evans' syndrome in Castleman's disease: unusual presentation with rectal bleeding from angiodysplasia of cecum. (1468898)
1992
43
Evans' syndrome as a presenting manifestation of atypical paroxysmal cold hemoglobinuria. (3578344)
1987
44
Evans' syndrome associated with microcystic adenoma of the pancreas. (3815308)
1987
45
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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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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Compounds related to Evans' Syndrome according to GeneCards Suite gene sharing:

idCompoundScoreTop Affiliating Genes
1rituximab43 49 1211.3HP, ADAMTS13
2urea43 24 1211.3HP, ADAMTS13
3endotoxin439.3HP, ADAMTS13
4nacl439.2HP, ADAMTS13
5fibrinogen439.2ADAMTS13, HP
6cyclosporin a43 28 5911.1HP, ADAMTS13
7steroid439.1HP, ADAMTS13
8creatinine439.0HP, ADAMTS13
9heparin43 28 24 1211.8HP, ADAMTS13

GO Terms for genes affiliated with Evans' Syndrome

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Cellular components related to Evans' Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:00056159.1HP, ADAMTS13

Products for genes affiliated with Evans' Syndrome

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies
  • Proteins
  • Kits and Assays

Sources for Evans' Syndrome

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet