MCID: FLT001
MIFTS: 64

Felty's Syndrome malady

Genetic diseases, Rare diseases, Bone diseases, Blood diseases categories
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Summaries for Felty's Syndrome

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NIH Rare Diseases:42 Felty's syndrome is a rare, potentially serious disorder that is defined by the presence of three conditions: rheumatoid arthritis (ra), an enlarged spleen (splenomegaly) and a decreased white blood cell count (neutropenia), which causes repeated infections. although some individuals with felty's syndrome are asymptomatic, others can develop serious and life-threatening infections. symptoms of felty's syndrome, in addition to those associated with the three conditions stated above, may include fatigue, fever, weight loss, discoloration of patches of skin, mild hepatomegaly (enlarged liver), lymphadenopathy (swelling of lymph nodes), sjögren syndrome, vasculitis, lower-extremity ulcers, and other findings. the exact cause is unknown, but several risk factors have been proposed, including autoimmunity. a few familial cases of the condition have been reported. treatment typically focuses on controlling the underlying ra; immunosuppressive therapy for ra may improve neutropenia and splenomegaly. last updated: 1/6/2011

MalaCards based summary: Felty's Syndrome, also known as felty syndrome, is related to neutropenia and arthritis, and has symptoms including subcutaneous nodules/lipomas/tumefaction/swelling, repeat respiratory infections and osteolysis/osteoclasia/bone destruction/erosions. An important gene associated with Felty's Syndrome is CSF2 (colony stimulating factor 2 (granulocyte-macrophage)), and among its related pathways are IL-15 Signaling and its Primary Biological Effects in Different Immune Cell Types and Staphylococcus aureus infection. The compounds bmmp and as 101 have been mentioned in the context of this disorder. Affiliated tissues include skin, liver and spleen, and related mouse phenotypes are tumorigenesis and hematopoietic system.

Disease Ontology:8 A syndrome that results in rheumatoid arthritis, splenomegaly and neutropenia.

Wikipedia:65 Felty\'s syndrome, also called Felty syndrome, is characterized by the combination of rheumatoid... more...

Description from OMIM:46 134750

Aliases & Classifications for Felty's Syndrome

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Sources:
8Disease Ontology, 42NIH Rare Diseases, 62UMLS, 48Orphanet, 46OMIM, 10DISEASES, 44Novoseek, 57SNOMED-CT, 27ICD9CM, 34MeSH, 39NCIt, 35MESH via Orphanet, 26ICD10 via Orphanet, 63UMLS via Orphanet, 25ICD10
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Felty's Syndrome, Aliases & Descriptions:

Name: Felty's Syndrome 8 42 62
Felty Syndrome 8 42 48 46
Familial Felty's Syndrome 42 62
Feltys Syndrome 10 44
 
Rheumatoid Arthritis with Splenoadenomegaly and Leukopenia 8
Splenomegaly-Neutropenia-Rheumatoid Arthritis Syndrome 48
Rheumatoid Arthritis, Splenomegaly and Neutropenia 42


Classifications:

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


Characteristics (Orphanet epidemiological data):

48
felty syndrome:
Age of onset: Adulthood; Age of death: Elderly


External Ids:

Disease Ontology8 DOID:11042
OMIM46 134750
ICD9CM27 714.1
MeSH34 D005258
NCIt39 C84712
SNOMED-CT57 156482001, 57160007
MESH via Orphanet35 D005258
ICD10 via Orphanet26 M05.0
UMLS via Orphanet63 C0015773
ICD1025 M05.0

Related Diseases for Felty's Syndrome

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

(show top 50)    (show all 65)
idRelated DiseaseScoreTop Affiliating Genes
1neutropenia30.1CSF3, IL3, CSF2
2arthritis30.0IL10, CSF2, IL8
3leukemia29.8EEF1A1, IL3, CSF2, CSF3, TRA
4rheumatoid arthritis29.8CSF2, TRA, IL10, IL8
5capillary leak syndrome10.5CSF2
6meningitis10.3IL8
7pdgfrb-associated chronic eosinophilic leukemia10.3CSF3, CSF2
8lung cancer10.2CSF3, CSF2
9stomatitis10.2CSF3, CSF2
10myelofibrosis10.2IL3, CSF3
11factor vii deficiency10.2CSF3, CSF2
12juvenile rheumatoid arthritis10.2
13large granular lymphocyte leukemia10.2
14pulmonary alveolar proteinosis10.2IL3, CSF2
15megakaryocytic leukemia10.2IL3, CSF2
16vernal keratoconjunctivitis10.1IL8, IL3
17acute graft versus host disease10.1IL10, CSF3
18monocytic leukemia10.1IL8, CSF2
19alcoholic hepatitis10.1IL10, IL8
20diphtheria10.1CSF2, IL3
21severe congenital neutropenia autosomal recessive 310.0CSF3, CSF2, IL3
22cyclic hematopoiesis10.0CSF3, IL3, CSF2
23histoplasmosis10.0
24leukemoid reaction10.0IL3, CSF2, CSF3
25severe congenital neutropenia10.0CSF2, IL3, CSF3
26pancytopenia10.0IL3, CSF2, CSF3
27refractory anemia10.0IL3, CSF3, CSF2
28thrombocytosis10.0CSF2, CSF3, IL3
29aplastic anemia10.0IL3, CSF2, CSF3
30leukopenia10.0IL3, CSF3, CSF2
31bone marrow cancer10.0CSF3, CSF2, IL3
32lupus nephritis10.0IL10, TRA
33thrombocytopenia10.0IL3, CSF2, CSF3
34hematologic cancer10.0IL3, CSF3, CSF2
35hodgkin's lymphoma10.0IL3, CSF3, CSF2
36chronic myeloid leukemia10.0CSF2, IL3, CSF3
37deficiency anemia10.0IL3, CSF2, CSF3
38non-hodgkin lymphoma10.0IL3, CSF2, CSF3
39acute leukemia10.0CSF3, CSF2, IL3
40acute lymphoblastic leukemia10.0IL3, CSF2, CSF3
41myeloid leukemia10.0CSF3, CSF2, IL3
42myeloma10.0CSF2, IL3, CSF3
43periodontitis10.0IL8, IL10
44tetanus9.9IL10, CSF2
45septic shock9.9CSF3, IL10, IL8
46acute promyelocytic leukemia9.9CSF3, IL3
47non-small cell lung carcinoma9.9CSF2, IL3, CSF3
48acute pancreatitis9.9IL8, IL10
49allergic rhinitis9.9IL8, IL10, CSF2
50ulcerative colitis9.9IL8, IL10, TRA

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



Diseases related to felty's syndrome

Symptoms for Felty's Syndrome

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Symptoms by clinical synopsis from OMIM:

134750

Clinical features from OMIM:

134750

Symptoms:

48 (show all 35)
  • subcutaneous nodules/lipomas/tumefaction/swelling
  • repeat respiratory infections
  • osteolysis/osteoclasia/bone destruction/erosions
  • articular/joint pain/arthralgia
  • arthritis/synovitis/synovial proliferation
  • restricted joint mobility/joint stiffness/ankylosis
  • joint/articular deformation
  • immunodeficiency/increased susceptibility to infections/recurrent infections
  • autoimmunity/autoimmune reaction/autoantibodies
  • polynuclear cells/neutrophils anomalies/neutropenia
  • nasal congestion/sinusitis/rhinitis/rhinorrhea
  • chronic/relapsing otitis
  • pharyngitis
  • splenomegaly
  • ground-glass/reticular/alveolar/interstitial lung/pulmonary opacity
  • lymphadenopathy/polyadenopathies
  • anaemia
  • lymphocytes anomalies
  • weight loss/loss of appetite/break in weight curve/general health alteration
  • chronic uveitis/blepharitis/episcleritis/scleritis/conjonctivitis/keratitis
  • cellulitis/panniculitis/pseudocellulitis/inflammation of subcutaneous tissue
  • chronic skin infection/ulcerations/ulcers/cancrum
  • diffuse/generalised skin hyperpigmentation/melanoderma
  • irregular/in bands/reticular skin hyperpigmentation
  • hepatomegaly/liver enlargement (excluding storage disease)
  • abnormal pleura/hydrothorax/pleuresia/pleural effusion/chylothorax
  • lung fibrosis
  • pericardium anomalies/pericarditis/absence/cysts/pericardial effusion
  • recurrent urinary infections
  • peripheral neuropathy
  • bone marrow failure/pancytopenia
  • thrombocytopenia/thrombopenia
  • lymphoma
  • early death/lethality
  • sepsis severe/septicemia

HPO human phenotypes related to Felty's Syndrome:

(show all 33)
id Description Frequency HPO Source Accession
1 arthritis hallmark (90%) HP:0001369
2 limitation of joint mobility hallmark (90%) HP:0001376
3 abnormality of neutrophils hallmark (90%) HP:0001874
4 osteolysis hallmark (90%) HP:0002797
5 arthralgia hallmark (90%) HP:0002829
6 autoimmunity hallmark (90%) HP:0002960
7 sinusitis typical (50%) HP:0000246
8 otitis media typical (50%) HP:0000388
9 splenomegaly typical (50%) HP:0001744
10 weight loss typical (50%) HP:0001824
11 anemia typical (50%) HP:0001903
12 lymphadenopathy typical (50%) HP:0002716
13 abnormality of lymphocytes typical (50%) HP:0004332
14 recurrent pharyngitis typical (50%) HP:0100776
15 recurrent urinary tract infections occasional (7.5%) HP:0000010
16 abnormality of the pericardium occasional (7.5%) HP:0001697
17 thrombocytopenia occasional (7.5%) HP:0001873
18 abnormality of the pleura occasional (7.5%) HP:0002103
19 pulmonary fibrosis occasional (7.5%) HP:0002206
20 hepatomegaly occasional (7.5%) HP:0002240
21 lymphoma occasional (7.5%) HP:0002665
22 bone marrow hypocellularity occasional (7.5%) HP:0005528
23 irregular hyperpigmentation occasional (7.5%) HP:0007400
24 generalized hyperpigmentation occasional (7.5%) HP:0007440
25 peripheral neuropathy occasional (7.5%) HP:0009830
26 inflammatory abnormality of the eye occasional (7.5%) HP:0100533
27 cellulitis occasional (7.5%) HP:0100658
28 sepsis occasional (7.5%) HP:0100806
29 skin ulcer occasional (7.5%) HP:0200042
30 autosomal dominant inheritance HP:0000006
31 rheumatoid arthritis HP:0001370
32 splenomegaly HP:0001744
33 neutropenia HP:0001875

Drugs & Therapeutics for Felty's Syndrome

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Drug clinical trials:

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Genetic Tests for Felty's Syndrome

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Anatomical Context for Felty's Syndrome

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MalaCards organs/tissues related to Felty's Syndrome:

32
Skin, Liver, Spleen, Lymph node, Bone, Neutrophil, Bone marrow, Lung, T cells, Myeloid, B cells, Endothelial

Animal Models for Felty's Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Felty's Syndrome:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020068.6CSF2, IL10, IL3, EEF1A1
2MP:00053977.2IL8, EEF1A1, IL3, IL10, CSF2, CSF3
3MP:00053877.0CSF3, EEF1A1, IL3, IL10, IL8, CSF2

Publications for Felty's Syndrome

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Articles related to Felty's Syndrome:

(show top 50)    (show all 332)
idTitleAuthorsYear
1
Felty's syndrome with hyperthyroidism: a case report and literature review. (24472276)
2014
2
Neutrophil activation and B-cell stimulation in the pathogenesis of Felty's syndrome. (22814518)
2012
3
Felty's syndrome as an initial presentation of rheumatoid arthritis: a case report. (19946450)
2009
4
Efficacy of rituximab in Felty's syndrome. (18697782)
2008
5
Felty's syndrome with chronic hepatitis and compatible autoimmune hepatitis: a case presentation. (15897648)
2005
6
Felty's syndrome. (15454123)
2004
7
Autoantibodies against granulocyte-macrophage colony stimulating factor and interleukin-3 are rare in patients with Felty's syndrome. (15194585)
2004
8
Autoantibodies against granulocyte colony-stimulating factor in Felty's syndrome and neutropenic systemic lupus erythematosus. (12355486)
2002
9
Major histocompatility complex haplotypic associations in Felty's syndrome and large granular lymphocyte syndrome are secondary to allelic association with HLA-DRB1 *0401. (10817772)
2000
10
Pulmonary veno-occlusive disease complicating Felty's syndrome. (9893784)
1998
11
Treatment of Felty's syndrome with the haemopoietic growth factor granulocyte colony-stimulating factor (G-CSF). (9519212)
1998
12
Interleukin-10 promoter polymorphisms in rheumatoid arthritis and Felty's syndrome. (9783765)
1998
13
Prolonged treatment with granulocyte colony-stimulating factor in a patient with Felty's syndrome and chronic renal failure from secondary amyloidosis. (9269662)
1997
14
Successful treatment of agranulocytosis and sepsis with granulocyte colony stimulating factor in a case of Felty's syndrome. (8523369)
1995
15
Felty's syndrome treated with rhG-CSF associated with flare of arthritis and skin rash. (7540527)
1995
16
Indication for and caution in the use of granulocyte colony-stimulating factor for the treatment of Felty's syndrome: comment on the report by Yasuda et al. (7524509)
1994
17
Orbital lymphoma in a patient with Felty's syndrome. (1540565)
1992
18
DR-DQ variants in rheumatoid arthritis and Felty's syndrome: comment on the article by Wallin et al. (1622429)
1992
19
Digital vasculitis after splenectomy in a patient with Felty's syndrome. (1632669)
1992
20
Analysis of lymphocyte phenotype and T cell receptor genotype in Felty's syndrome. (1380988)
1992
21
Effect of corticosteroid treatment on hemopoiesis in vivo and in vitro in a patient with Felty's syndrome. (1786345)
1991
22
Ectopic splenomegaly in Felty's syndrome. (2032427)
1991
23
Worsening of Felty's syndrome with methotrexate. (1929594)
1991
24
Is there a place for splenectomy in the treatment of Felty's syndrome? (2306569)
1990
25
Resolution of Felty's syndrome with cyclophosphamide. (2754676)
1989
26
Felty's syndrome: a therapeutic dilemma? (2769662)
1989
27
Neutropenia and infections in Felty's syndrome. (3378123)
1988
28
Felty's syndrome in black Americans: a case report. (3594967)
1987
29
Simultaneous presentation of rheumatoid arthritis and acute myeloid leukaemia masquerading as Felty's syndrome. (3465419)
1986
30
Radiocolloid scintigraphy in Felty's syndrome. (3487213)
1986
31
Treatment of Felty's syndrome with low-dose oral methotrexate. (3741502)
1986
32
HLA-DR4 in Felty's syndrome. (3876440)
1985
33
Felty's syndrome in an Asian. (4016489)
1985
34
D-penicillamine in Felty's syndrome. (4057191)
1985
35
Felty's syndrome in a child. (6520839)
1984
36
Kingella kingae causing septic arthritis in Felty's syndrome. (6622347)
1983
37
High-dose immunoglobulin in Felty's syndrome. (6882047)
1983
38
HLA-DRw4 in Felty's syndrome. (6934775)
1980
39
Felty's syndrome: granulocyte-bound immunoglobulin G and splenectomy. (970768)
1976
40
Letter: Lithium in Felty's syndrome. (1259276)
1976
41
Familial Felty's syndrome. (984910)
1976
42
Spontaneous remission of Felty's syndrome. (1191353)
1975
43
Splenectomy and Felty's syndrome. (1146350)
1975
44
The mechanism of neutropenia in Felty's syndrome. (4416356)
1974
45
Symptomatic porphyria in a case of Felty's syndrome. I. Clinical and routine biochemcial studies. (5019121)
1972
46
Symptomatic porphyria in a case of Felty's syndrome. II. Biochemical investigations. (5019122)
1972
47
Leucocyte-specific anti-nuclear factors in patients with felty's syndrome, rheumatoid arthritis, systemic lupus erythematosus and other diseases. (5325973)
1966
48
Felty's syndrome; response to splenectomy after cortisone failure. (13227656)
1955
49
Neutropenia associated with splenomegaly and atrophic arthritis (Felty's syndrome); report of a case in which splenectomy was performed. (15411128)
1950
50
Primary splenic neutropenia with rheumatoid arthritis. (Felty's syndrome). (15427053)
1950

Variations for Felty's Syndrome

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Expression for genes affiliated with Felty's Syndrome

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Expression patterns in normal tissues for genes affiliated with Felty's Syndrome

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Pathways for genes affiliated with Felty's Syndrome

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Pathways related to Felty's Syndrome according to GeneCards/GeneDecks:

(show all 22)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.7IL10, CSF2
29.6IL10, C4A
39.6CSF2, IL8
49.6IL8, CSF2
59.3IL8, EEF1A1
6
Show member pathways
9.2IL3, C4A, IL10
79.2CSF2, IL3, IL8
8
Show member pathways
Calcium signaling in the CD4+ TCR pathway37
9.2IL8, CSF2, IL3
99.2CSF2, IL10, IL8
10
Show member pathways
BCR signaling pathway37
TCR Signaling Pathway37
9.2TRA, CSF2, IL10
119.1IL10, IL8, C4A
129.1IL10, IL8, C4A
139.1IL3, IL8, IL10
148.9IL3, CSF3, CSF2
15
Show member pathways
8.8CSF2, IL8, CSF3
168.7IL8, IL10, CSF3
17
Show member pathways
Type III interferon signaling37
8.4CSF3, CSF2, IL3, IL10
188.3TRA, IL10, CSF2, CSF3
19
Show member pathways
7.9CSF3, CSF2, IL8, IL10, IL3
20
Show member pathways
7.9IL8, IL3, IL10, CSF2, CSF3
21
Show member pathways
7.9IL10, IL8, CSF2, IL3, CSF3
22
Show member pathways
7.9CSF2, IL8, CSF3, IL10, IL3

Compounds for genes affiliated with Felty's Syndrome

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Compounds related to Felty's Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 140)
idCompoundScoreTop Affiliating Genes
1bmmp4410.0IL3, CSF2
2as 10144 6110.6IL10, IL3, CSF2
3azithromycin44 1110.5IL8, CSF2, IL10
4lenograstim449.5CSF3, IL3
5il-12449.5CSF2, IL10, IL8
6imiquimod44 61 28 1112.5CSF2, IL8, IL10
7groalpha449.5CSF2, IL8, IL10
8romurtide449.5CSF3, CSF2
9poly(i-c)449.5IL3, IL8, CSF2
10ivig449.4CSF2, IL10, IL3
11molgramostim449.3CSF3, CSF2, IL3
12pegfilgrastim44 1110.3IL3, CSF2, CSF3
13mesna449.3CSF2, CSF3, IL3
14quinine44 28 2 50 1113.3IL10, CSF2, IL3
15tarc449.3CSF2, IL8, IL10
16thiotepa44 50 1111.3IL3, CSF2, CSF3
17ara-ctp449.3CSF3, CSF2, IL3
18mafosfamide449.3CSF3, IL3, CSF2
194-hydroperoxycyclophosphamide449.3CSF3, CSF2, IL3
20pixy321449.3CSF2, IL3, CSF3
21amifostine44 1110.3IL3, CSF3, CSF2
22filgrastim44 1110.3CSF3, IL3, CSF2
23anaphylatoxin449.3CSF2, IL3, IL8, C4A
24idarubicin50 44 1111.3IL3, CSF3, CSF2
25ifosfamide44 1110.2CSF2, IL3, CSF3
26mip 1alpha449.2CSF2, IL8, IL10, IL3
27il 10449.2IL8, CSF2, IL3, IL10
28mitoxantrone44 50 1111.2IL3, CSF2, CSF3
29neopterin449.2CSF2, IL3, IL8, IL10
30methylcellulose449.1CSF2, IL3, CSF3
31hydrocortisone44 2 61 1112.1CSF2, IL10, IL3, IL8
32leucovorin44 50 1111.1CSF2, IL3, CSF3
33ionomycin449.1TRA, IL10, IL3, CSF2
34pentoxifylline44 1110.1CSF2, IL8, IL10
35mycophenolate mofetil44 50 1111.0IL3, IL10, CSF3
36melphalan44 50 1110.9CSF3, CSF2, IL3
37ribavirin44 50 1110.9IL3, IL10, CSF2, CSF3
38zidovudine44 50 1110.8IL10, CSF2, CSF3, IL3
39hydroxyurea44 50 1110.8IL10, IL3, CSF2, CSF3
40n-formylmethionyl-leucylphenylalanine448.8CSF2, CSF3, IL3, IL8
41cyclophosphamide44 50 1110.8CSF2, CSF3, IL3, IL10
42fmlp448.7CSF3, IL3, CSF2, IL8
43ccl344 289.7CSF3, IL8, IL10
44histamine44 28 2410.4IL3, IL10, IL8, CSF2
45endotoxin448.4CSF3, CSF2, C4A, IL10, IL8
46gm-csf44 289.4IL10, IL8, IL3, CSF2, CSF3
47rantes448.4IL10, IL8, CSF2, CSF3, IL3
48cyclosporin a44 28 6110.3IL10, IL8, CSF2, CSF3, IL3
49cycloheximide448.3IL8, IL10, IL3, CSF3, CSF2
50dexamethasone44 50 28 1111.2IL8, IL10, CSF3, IL3, CSF2

GO Terms for genes affiliated with Felty's Syndrome

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Cellular components related to Felty's Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular regionGO:0055768.8IL3, IL8, CSF2, C4A
2extracellular spaceGO:0056157.0EEF1A1, IL3, IL10, IL8, CSF2, CSF3

Biological processes related to Felty's Syndrome according to GeneCards/GeneDecks:

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of tyrosine phosphorylation of Stat5 proteinGO:0425239.8IL3, CSF2
2positive regulation of DNA replicationGO:0457409.6IL3, CSF2
3response to molecule of bacterial originGO:0022379.6IL8, IL10
4positive regulation of peptidyl-tyrosine phosphorylationGO:0507319.2CSF3, IL3
5inflammatory responseGO:0069549.1C4A, IL8, IL10
6positive regulation of cell proliferationGO:0082848.6CSF3, CSF2, IL3
7immune responseGO:0069558.5IL3, IL8, CSF2, CSF3
8cellular response to lipopolysaccharideGO:0712228.5CSF3, CSF2, IL8, IL10

Molecular functions related to Felty's Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cytokine activityGO:0051258.4CSF3, CSF2, IL10, IL3
2growth factor activityGO:0080838.1CSF3, CSF2, IL10, IL3

Products for genes affiliated with Felty's Syndrome

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Sources for Felty's 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