MCID: FLT001
MIFTS: 62

Felty's Syndrome malady

Bone diseases, Blood diseases, Genetic diseases categories

Summaries for Felty's Syndrome

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8Disease Ontology, 42NIH Rare Diseases, 63Wikipedia, 46OMIM, 32MalaCards
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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: Felty's Syndrome, also known as felty syndrome, is related to arthritis and leukemia, and has symptoms including repeat respiratory infections, chronic uveitis/blepharitis/episcleritis/scleritis/conjonctivitis/keratitis and polynuclear cells/neutrophils anomalies/neutropenia. An important gene associated with Felty's Syndrome is C4A (complement component 4A (Rodgers blood group)), and among its related pathways are Rho Family GTPases and Cytokines and Inflammatory Response. The compounds gm-csf and hydrocortisone have been mentioned in the context of this disorder. Affiliated tissues include skin, liver and spleen, and related mouse phenotypes are immune system and hematopoietic system.

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

Wikipedia:63 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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8Disease Ontology, 42NIH Rare Diseases, 60UMLS, 48Orphanet, 46OMIM, 10DISEASES, 44Novoseek, 56SNOMED-CT, 39NCIt, 27ICD9CM, 34MeSH, 35MESH via Orphanet, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet, 25ICD10
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Classifications:

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


Characteristics (Orphanet epidemiological data):

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


Aliases & Descriptions:

felty's syndrome 8 42 60
felty syndrome 42 48 46
familial felty's syndrome 42 60
feltys syndrome 10 44
rheumatoid arthritis with splenoadenomegaly and leukopenia 8
splenomegaly-neutropenia-rheumatoid arthritis syndrome 48
rheumatoid arthritis, splenomegaly and neutropenia 42


External Ids:

Disease Ontology8 DOID:11042
NCIt39 C84712
ICD9CM27 714.1
MeSH34 D005258
OMIM46 134750
SNOMED-CT56 156482001, 57160007
MESH via Orphanet35 D005258
ICD10 via Orphanet26 M05.0
SNOMED-CT via Orphanet57 57160007
UMLS via Orphanet61 C0015773
ICD1025 M05.0

Related Diseases for Felty's Syndrome

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17GeneCards, 18GeneDecks
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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 97)
idRelated DiseaseScoreTop Affiliating Genes
1arthritis30.2IL8, IL10, CSF2
2leukemia30.1CSF2, CSF3, TRA, IL3, B3GAT1, EEF1A1
3rheumatoid arthritis30.1CSF2, TRA, IL10, IL8, B3GAT1
4neutropenia29.9CSF2, CSF3, IL3, B3GAT1
5juvenile rheumatoid arthritis10.1
6natural killer cell leukemia10.0
7adult syndrome10.0
8histoplasmosis10.0
9hepatitis10.0EEF1A1
10capillary leak syndrome10.0CSF2
11prostate cancer10.0EEF1A1
12lupus erythematosus10.0C4A
13multiple myeloma10.0CSF3
14hypereosinophilic syndrome10.0CSF2
15glycogen storage disease10.0CSF3
16dermatitis10.0CSF2
17severe combined immunodeficiency10.0IL3
18sarcoma10.0B3GAT1
19human immunodeficiency virus infectious disease10.0B3GAT1
20astrocytoma10.0B3GAT1
21kidney cancer10.0CSF2
22polycythemia vera10.0IL3
23polycythemia10.0IL3
24periodontitis10.0IL8
25bronchiolitis10.0IL8
26rhinitis10.0IL8
27pulmonary fibrosis10.0IL8
28pulmonary eosinophilia10.0IL8
29cystic fibrosis10.0IL8
30gastritis10.0IL8
31chorioamnionitis10.0IL8
32meningitis10.0IL8
33stomatitis10.0CSF2, CSF3
34endometriosis10.0IL8
35malaria10.0IL10
36lung cancer10.0CSF3, CSF2
37hypersensitivity reaction type ii disease10.0IL10
38pulmonary alveolar proteinosis10.0CSF2, IL3
39hepatitis c10.0IL10
40atopy10.0IL10
41myelofibrosis10.0IL3, CSF3
42galactosemia10.0IL10
43common variable immune deficiency10.0IL10
44chronic myelomonocytic leukemia10.0CSF3, CSF2
45pancreatic cancer10.0IL8
46germ cell cancer10.0B3GAT1
47megakaryocytic leukemia10.0IL3, CSF2
48tuberculosis10.0EEF1A1, IL10
49monocytic leukemia10.0IL8, CSF2
50factor vii deficiency10.0CSF3, CSF2

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



Diseases related to felty's syndrome

Clinical Features for Felty's Syndrome

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46OMIM, 48Orphanet
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Clinical features from OMIM:

134750

Clinical synopsis from OMIM:

134750

Symptoms:

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

Drugs & Therapeutics for Felty's Syndrome

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

Search CenterWatch for Felty's Syndrome

Drug clinical trials:

Search ClinicalTrials for Felty's Syndrome

Search NIH Clinical Center for Felty's Syndrome

Search CenterWatch for Felty's Syndrome

Genetic Tests for Felty's Syndrome

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Anatomical Context for Felty's Syndrome

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Sources:
32MalaCards
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MalaCards organs/tissues related to Felty's Syndrome:

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

Animal Models for Felty's Syndrome or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Felty's Syndrome:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053877.3CSF3, IL3, IL10, IL8, EEF1A1, CSF2
2MP:00053977.0EEF1A1, IL8, IL10, IL3, CSF3, CSF2

Publications for Felty's Syndrome

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Sources:
50PubMed
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Articles related to Felty's Syndrome:

(show top 50)    (show all 334)
idTitleAuthorsYear
1
The spectrum of large granular lymphocyte leukemia and Felty's syndrome. (21546829)
2011
2
Felty's syndrome and Kala-azar: a challenge for the rheumatologist. (21243309)
2010
3
Felty's syndrome with chronic hepatitis and compatible autoimmune hepatitis: a case presentation. (15897648)
2005
4
Treatment of Felty's syndrome with leflunomide. (11327265)
2001
5
Cloning and expression of a novel human antibody-antigen pair associated with Felty's syndrome. (10922075)
2000
6
Pulmonary veno-occlusive disease complicating Felty's syndrome. (9893784)
1998
7
Felty's syndrome, pseudo-felty's syndrome, monoclonal or polyclonal CD3 lymphocytosis of undetermined significance. (9064110)
1996
8
Pseudo-felty's syndrome. Report of a case with no symptoms for at least 15 years. (9064112)
1996
9
Sustained response in Felty's syndrome to prolonged administration of recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF). (7778576)
1995
10
The role of interleukin-8 and other cytokines in the pathogenesis of Felty's syndrome. (7554553)
1995
11
Neutropenia of Felty's syndrome successfully treated with granulocyte colony stimulating factor. (7693942)
1993
12
Treatment of neutropenia in Felty's syndrome with granulocyte-macrophage colony-stimulating factor--hematological response accompanied by pulmonary complications with lethal outcome. (1457582)
1992
13
Cyclosporine for the treatment of granulocytopenia in Felty's syndrome. (1996561)
1991
14
Felty's syndrome: response to cyclosporin A with disappearance of neutrophil autoantibodies. (1994932)
1991
15
The clinical course of Felty's syndrome compared to matched controls. (1941816)
1991
16
Granulocyte-macrophage colony-stimulating factor treatment of neutropenia associated with Felty's syndrome. (2261008)
1990
17
DQw7 and the C4B null allele in rheumatoid arthritis and Felty's syndrome. (2270969)
1990
18
Felty's syndrome without concomitant arthritis: a variant. (2758710)
1989
19
Felty's syndrome in black Americans: a case report. (3594967)
1987
20
Simultaneous presentation of rheumatoid arthritis and acute myeloid leukaemia masquerading as Felty's syndrome. (3465419)
1986
21
Radiocolloid scintigraphy in Felty's syndrome. (3487213)
1986
22
The inheritance of Felty's syndrome in a family with several affected members. (3701742)
1986
23
Felty's syndrome and HLA-DR antigens. (3486496)
1986
24
A possible haplotype association in Felty's syndrome. (3502561)
1986
25
Cyclosporine in Felty's syndrome. (3941701)
1986
26
Felty's syndrome in a child. (6520839)
1984
27
C3 receptors on granulocytes from patients with rheumatoid arthritis and Felty's syndrome. (6380835)
1984
28
Felty's syndrome presenting without arthritis. (6416525)
1983
29
Non-articular Felty's syndrome. (7063810)
1982
30
Increased endothelial cell adherence, aggregation, and superoxide generation by neutrophils incubated in systemic lupus erythematosus and Felty's syndrome sera. (6293513)
1982
31
Failure of splenectomy in Felty's syndrome. The role of antibodies supporting granulocyte lysis by lymphocytes. (7453725)
1981
32
HLA-DRw4 in Felty's syndrome. (6934775)
1980
33
Pasteurella multocida septicaemia complicating Felty's syndrome: A case report. (7404234)
1980
34
The rarity of Felty's syndrome in blacks. (475875)
1979
35
Familial Felty's syndrome. (984910)
1976
36
Cryoimmunoglobulinemia in Felty's syndrome. (1082749)
1976
37
Defective responsiveness to ascorbic acid of neutrophil random and chemotactic migration in Felty's syndrome and systemic lupus erythematosus. (1008618)
1976
38
Felty's syndrome. (4547210)
1974
39
Symptomatic porphyria in a case of Felty's syndrome. II. Biochemical investigations. (5019122)
1972
40
Felty's syndrome. A clinical and pathological survey of 21 patients and their response to treatment. (4104268)
1971
41
Liver in Felty's syndrome. (5411618)
1970
42
Characteristics of antinuclear factors in Felty's syndrome. (4191420)
1970
43
The role of splenectomy in Felty's syndrome. (5635187)
1968
44
Felty's syndrome. Good response to adrenocorticosteroids: possible mechanism of the anaemia. (5921743)
1966
45
Splenic neutropenia in Felty's syndrome. (13472060)
1957
46
Felty's syndrome. (13311223)
1956
47
Pancytopenia associated with rheumatoid arthritis; Felty's syndrome; treatment with cortisone and splenectomy. (13222068)
1954
48
Felty's Syndrome Treated by Splenectomy. (20788189)
1953
49
Ulcers of the leg in Felty's syndrome. (14952027)
1952
50
Rheumatoid arthritis with neutropenia, thrombocytopenia, and splenomegaly (Felty's syndrome) with improvement after splenectomy. (14787946)
1950

Genetic Variations for Felty's Syndrome

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Expression for genes affiliated with Felty's Syndrome

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Felty's Syndrome

Search GEO for disease gene expression data for Felty's Syndrome.

Pathways for genes affiliated with Felty's Syndrome

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51QIAGEN, 37NCBI BioSystems Database, 52R&D Systems, 29KEGG
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Pathways related to Felty's Syndrome according to GeneCards/GeneDecks:

(show all 17)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
10.1CSF2
210.0TRA
39.7IL8, CSF2
4
Hide members
9.6IL10, CSF2
5
Hide members
9.3CSF2, IL3, IL8
69.3IL8, IL3, CSF2
79.2IL8, IL10, C4A
89.2C4A, IL10, IL8
99.2IL8, IL10, CSF2
109.1IL3, IL10, IL8
11
Hide members
9.1CSF2, TRA, IL10
128.9CSF2, CSF3, IL3
13
Cytokine production by Th17 cells in CF (Mouse model)
8.9CSF2, CSF3, IL8
148.4IL10, IL3, CSF3, CSF2
15
Hide members
8.0CSF2, CSF3, IL3, IL10, IL8
16
Hide members
8.0CSF2, CSF3, IL3, IL10, IL8
17
Hide members
8.0CSF2, CSF3, IL3, IL10, IL8

Compounds for genes affiliated with Felty's Syndrome

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Sources:
44Novoseek, 28IUPHAR, 2BitterDB, 59Tocris Bioscience, 11DrugBank, 49PharmGKB, 24HMDB
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Compounds related to Felty's Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 118)
idCompoundScoreTop Affiliating Genes
1gm-csf44 2811.4IL10
2hydrocortisone44 2 59 1113.4IL8
3bmmp4410.1CSF2, IL3
4endotoxin449.8C4A, CSF3
5as 10159 4410.6CSF2, IL3, IL10
6lenograstim449.6CSF3, IL3
7romurtide449.6CSF2, CSF3
8poly(i-c)449.6CSF2, IL3, IL8
9azithromycin44 1110.5IL8, IL10, CSF2
10il-12449.5IL8, IL10, CSF2
11imiquimod44 28 59 1112.5IL8, IL10, CSF2
12groalpha449.5IL8, IL10, CSF2
13daniplestim449.4IL3, CSF3
14anaphylatoxin449.4CSF2, C4A, IL3, IL8
15molgramostim449.4CSF2, CSF3, IL3
16pegfilgrastim44 1110.4IL3, CSF3, CSF2
17mesna449.4CSF2, CSF3, IL3
18thiotepa44 49 1111.4CSF2, CSF3, IL3
19ara-ctp449.4CSF2, CSF3, IL3
20mafosfamide449.4CSF2, CSF3, IL3
21rantes449.4CSF2, CSF3, IL3
22ivig449.3CSF2, IL3, IL10
234-hydroperoxycyclophosphamide449.3CSF2, CSF3, IL3
24pixy321449.3IL3, CSF3, CSF2
25amifostine44 1110.3CSF2, CSF3, IL3
26tarc449.3IL8, IL10, CSF2
27idarubicin44 49 1111.3IL3, CSF3, CSF2
28filgrastim44 1110.3IL3, CSF3, CSF2
29quinine44 28 2 1112.3IL10, IL3, CSF2
30mip 1alpha449.2IL8, IL10, IL3, CSF2
31ifosfamide44 1110.2IL3, CSF3, CSF2
32leucovorin44 49 1111.2IL3, CSF3, CSF2
33il 10449.2IL8, IL10, IL3, CSF2
34neopterin449.2IL8, IL10, IL3, CSF2
35mitoxantrone44 49 1111.2CSF2, CSF3, IL3
36methylcellulose449.1CSF2, CSF3, IL3
37pentoxifylline44 1110.1CSF2, IL10, IL8
38ionomycin449.0CSF2, TRA, IL3, IL10
39mycophenolate mofetil44 49 1111.0CSF3, IL3, IL10
40melphalan44 119.9IL3, CSF3, CSF2
41n-formylmethionyl-leucylphenylalanine448.9CSF2, CSF3, IL3, IL8
42ribavirin44 49 1110.8CSF2, CSF3, IL3, IL10
43zidovudine44 119.8IL10, IL3, CSF3, CSF2
44fmlp448.8CSF2, CSF3, IL3, IL8
45hydroxyurea44 49 1110.8CSF2, CSF3, IL3, IL10
46histamine44 28 2410.8CSF2, IL3, IL10, IL8, B3GAT1
47cyclophosphamide44 49 1110.8CSF2, CSF3, IL3, IL10
48ccl344 289.7CSF3, IL10, IL8
49cyclosporin a44 28 5910.3CSF2, CSF3, IL3, IL10, IL8
50dexamethasone44 49 28 1110.7CSF2, CSF3, IL3, IL10, IL8

GO Terms for genes affiliated with Felty's Syndrome

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16Gene Ontology
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Cellular components related to Felty's Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1extracellular spaceGO:0056157.6IL8, IL10, IL3, C4A, CSF3, CSF2

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

idNameGO IDScoreTop Affiliating Genes
1positive regulation of DNA replicationGO:0457409.8IL3, CSF2
2positive regulation of tyrosine phosphorylation of Stat5 proteinGO:0425239.5IL3, CSF2
3response to molecule of bacterial originGO:0022379.4IL8, IL10
4immune responseGO:0069558.5CSF2, CSF3, IL3, IL8
5cellular response to lipopolysaccharideGO:0712228.4IL8, IL10, CSF3, CSF2

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

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

Products for genes affiliated with Felty's Syndrome

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

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
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet