MCID: FLT001
MIFTS: 47

Felty's Syndrome malady

Genetic diseases, Rare diseases, Bone diseases, Blood diseases categories

Aliases & Classifications for Felty's Syndrome

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Sources:
10Disease Ontology, 45NIH Rare Diseases, 12DISEASES, 51Orphanet, 65UMLS, 36MeSH, 47Novoseek, 59SNOMED-CT, 42NCIt, 29ICD9CM, 28ICD10 via Orphanet, 37MESH via Orphanet, 66UMLS via Orphanet, 27ICD10
See all sources

Aliases & Descriptions for Felty's Syndrome:

Name: Felty's Syndrome 10 45 12
Felty Syndrome 10 45 51 65 36
Familial Felty's Syndrome 45 65
Rheumatoid Arthritis with Splenoadenomegaly and Leukopenia 10
 
Splenomegaly-Neutropenia-Rheumatoid Arthritis Syndrome 51
Rheumatoid Arthritis, Splenomegaly and Neutropenia 45
Feltys Syndrome 47


Classifications:



Characteristics (Orphanet epidemiological data):

51
felty syndrome:
Age of onset: Adult; Age of death: elderly


External Ids:

Disease Ontology10 DOID:11042
NCIt42 C84712
MeSH36 D005258
ICD9CM29 714.1
Orphanet51 47612
SNOMED-CT59 57160007, 156482001
ICD10 via Orphanet28 M05.0
MESH via Orphanet37 D005258
UMLS via Orphanet66 C0015773
ICD1027 M05.0

Summaries for Felty's Syndrome

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NIH Rare Diseases:45 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 rheumatoid arthritis and arthritis, and has symptoms including arthritis, limitation of joint mobility and abnormality of neutrophils. An important gene associated with Felty's Syndrome is C4A (Complement Component 4A (Rodgers Blood Group)), and among its related pathways are Cytokines and Inflammatory Response and Pertussis. Affiliated tissues include skin, liver and bone, and related mouse phenotypes are hematopoietic system and immune system.

Disease Ontology:10 A syndrome that results in rheumatoid arthritis, splenomegaly and neutropenia.

Wikipedia:68 Felty\'s syndrome, also called Felty syndrome, is characterized by the combination of rheumatoid... more...

Related Diseases for Felty's Syndrome

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

(show top 50)    (show all 77)
idRelated DiseaseScoreTop Affiliating Genes
1rheumatoid arthritis10.2
2arthritis10.2
3leukemia10.2
4juvenile rheumatoid arthritis10.2
5large granular lymphocyte leukemia10.2
610p13-p14 deletion syndrome10.2CSF3, ELANE
7gingival overgrowth10.1ELANE, LTF
8acute diarrhea10.1CXCL8, LTF
9papilledema10.1CXCL8, LTF
10cicatricial entropion10.1CXCL8, LTF
11hair defect with photosensitivity and mental retardation10.1CXCL8, LTF
12pancreatic vasoactive intestinal peptide producing tumor10.1CXCL8, LTF
13acute female pelvic peritonitis10.1CXCL8, LTF
14neutropenia10.1
15histoplasmosis10.1
16cardioauditory syndrome of sanchez cascos10.1CXCL8, ELANE
17chronic mucocutaneous candidiasis10.1CXCL8, LTF
18scotoma10.1CXCL8, ELANE
19enolase deficiency type 110.0CSF2, CSF3
20hyperinsulinemic hypoglycemia10.0CXCL8, LTF
21functionless pituitary adenoma10.0CSF2, CSF3
22pasteurellosis10.0CXCL8, LTF
23amelogenesis imperfecta10.0CXCL8, ELANE
24mature b-cell neoplasm10.0CXCL8, LTF
25bardet-biedl syndrome 810.0CXCL8, ELANE
26epiglottitis10.0CSF2, CSF3
27burn scar10.0CXCL8, ELANE
28connective tissue benign neoplasm10.0CXCL8, LTF
29acute zonal occult outer retinopathy10.0CXCL8, ELANE
30mixed cerebral palsy10.0CXCL8, ELANE
31cyclic vomiting syndrome10.0CSF2, IL3
32ileum cancer10.0CXCL8, ELANE
33keratosis10.0CXCL8, LTF
34ependymoblastoma10.0CSF2, IL3
35chancroid10.0CSF2, CSF3
36leukemia, acute promyelocytic, somatic10.0CSF3, ELANE, IL3
37wild type abeta2m amyloidosis9.9CSF2, CXCL8
38acute mountain sickness9.9CSF2, CXCL8
39melanoma metastasis9.9CSF2, IL3
40echinostomiasis9.9CSF2, CXCL8
41vulvovaginal candidiasis9.9CSF2, CXCL8
42strabismus9.9CSF2, CSF3
43pneumothorax9.9CSF3, CXCL8, ELANE
44bronchial neoplasm9.9CXCL8, ELANE
45adult t-cell leukemia9.9C4A, CXCL8, ELANE
46trigeminal neuralgia9.9CSF2, IL3
47glioblastoma multiforme9.9CXCL8, ELANE, LTF
48large cell keratinizing variant squamous cell breast carcinoma9.9CXCL8, ELANE, LTF
49periodontitis9.9CXCL8, ELANE, LTF
50wegener granulomatosis9.9CXCL8, ELANE, LTF

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:

 51 (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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Drugs for Felty's Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
Cyclophosphamideapproved, investigationalPhase 2252550-18-0, 6055-19-22907
Synonyms:
(+-)-Cyclophosphamide
(-)-Cyclophosphamide
(RS)-Cyclophosphamide
1-(bis(2-chloroethyl)amino)-1-oxo-2-aza-5-oxaphosphoridine
1-Bis(2-chloroethyl)amino-1-oxo-2-aza-5-oxaphosphoridin
2-[Bis(2-chloroethylamino)]-tetrahydro-2H-1,3,2-oxazaphosphorine-2-oxide
4-Hydroxy-cyclophosphan-mamophosphatide
50-18-0
60007-95-6
6055-19-2 (monohydrate)
75526-90-8
AC1L1EQQ
AI3-26198
ASTA
ASTA B518
Asta B 518
B 518
B-518
BRN 0011744
BSPBio_002099
Bis(2-chloroethyl)phosphoramide cyclic propanolamide ester
C 0768
C07888
C7H15Cl2N2O2P
CB 4564
CB-4564
CCRIS 188
CHEBI:4027
CHEMBL32520
CHEMBL88
CID2907
CP
CPA
CTX
CY
Ciclofosfamida
Ciclofosfamida [INN-Spanish]
Ciclofosfamide
Ciclophosphamide
Ciclophosphamide [INN]
Clafen
Claphene
Cycloblastin
Cyclophosphamid
Cyclophosphamide
Cyclophosphamide (INN)
Cyclophosphamide (TN)
Cyclophosphamide (anhydrous form)
Cyclophosphamide (anhydrous)
Cyclophosphamide Monohydrate
Cyclophosphamide Sterile
Cyclophosphamide anhydrous
Cyclophosphamide, (+-)-Isomer
Cyclophosphamides
Cyclophosphamidum
Cyclophosphamidum [INN-Latin]
Cyclophosphan
Cyclophosphane
Cyclophosphanum
Cyclophosphoramide
Cyclostin
Cyklofosfamid
Cyklofosfamid [Czech]
Cytophosphan
Cytophosphane
Cytoxan
Cytoxan (TN)
Cytoxan Lyoph
D,L-Cyclophosphamide
D07760
DB00531
 
DivK1c_000246
EINECS 200-015-4
EU-0100238
Endoxan
Endoxan R
Endoxan-Asta
Endoxana
Endoxanal
Endoxane
Enduxan
Genoxal
HMS2090A12
HSDB 3047
Hexadrin
IDI1_000246
KBio1_000246
KBio2_001338
KBio2_003906
KBio2_006474
KBio3_001319
KBioGR_000888
KBioSS_001338
LS-1302
LS-99787
Ledoxina
Lopac-C-0768
Lopac0_000238
Lyophilized Cytoxan
Mitoxan
MolPort-001-783-420
N,N-Bis(2-chloroethyl)-1,3,2-oxazaphosphinan-2-amine 2-oxide
N,N-Bis(2-chloroethyl)tetrahydro-2H-1,3,2-oxazaphosphorin-2-amine 2-oxide
NCGC00015209-01
NCGC00015209-03
NCGC00015209-06
NCGC00091741-02
NCGC00091741-03
NCI-C04900
NCI60_002097
NINDS_000246
NSC 26271
NSC-26271
NSC26271
NSC273033
NSC273034
Neosar
Occupation, cyclophosphamide exposure
Procytox
RCRA waste no. U058
Rcra Waste Number U058
Rcra waste number U058
Revimmune
S1217_Selleck
SK 20501
SPBio_001071
STK177249
STOCK2S-91217
Semdoxan
Sendoxan
Senduxan
Spectrum2_001146
Spectrum3_000370
Spectrum4_000304
Spectrum5_000795
Spectrum_000858
UNII-6UXW23996M
WLN: T6MPOTJ BO BN2G2G
Zyklophosphamid
Zyklophosphamid [German]
bis(2-Chloroethyl)phosphami de cyclic propanolamide
bis(2-Chloroethyl)phosphamide cyclic propanolamide ester
cyclophosphamide
2lenograstimPhase 21108

Interventional clinical trials:

idNameStatusNCT IDPhase
1Phase II Study of High-Dose Cyclophosphamide in Patients With Severe Autoimmune Hematologic DiseaseCompletedNCT00010387Phase 2
2Use of Well Known Drugs for New Destination - RA Improvement (RANT)CompletedNCT02633332Phase 1

Search NIH Clinical Center for Felty's Syndrome


Cochrane evidence based reviews: Felty Syndrome

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:

33
Skin, Liver, Bone, Spleen, Lymph node, Neutrophil, Bone marrow

Animal Models for Felty's Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Felty's Syndrome:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053977.1CSF2, CSF3, CXCL8, ELANE, IL3, LTF
2MP:00053876.8CSF2, CSF3, CXCL8, ELANE, IL3, LTF

Publications for Felty's Syndrome

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Articles related to Felty's Syndrome:

(show top 50)    (show all 336)
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
Neutrophil phagocytosis in Felty's syndrome. (11705439)
2001
10
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
11
Pulmonary veno-occlusive disease complicating Felty's syndrome. (9893784)
1998
12
Treatment of Felty's syndrome with the haemopoietic growth factor granulocyte colony-stimulating factor (G-CSF). (9519212)
1998
13
Interleukin-10 promoter polymorphisms in rheumatoid arthritis and Felty's syndrome. (9783765)
1998
14
Prolonged treatment with granulocyte colony-stimulating factor in a patient with Felty's syndrome and chronic renal failure from secondary amyloidosis. (9269662)
1997
15
Successful treatment of agranulocytosis and sepsis with granulocyte colony stimulating factor in a case of Felty's syndrome. (8523369)
1995
16
Felty's syndrome treated with rhG-CSF associated with flare of arthritis and skin rash. (7540527)
1995
17
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
18
Orbital lymphoma in a patient with Felty's syndrome. (1540565)
1992
19
DR-DQ variants in rheumatoid arthritis and Felty's syndrome: comment on the article by Wallin et al. (1622429)
1992
20
Digital vasculitis after splenectomy in a patient with Felty's syndrome. (1632669)
1992
21
Analysis of lymphocyte phenotype and T cell receptor genotype in Felty's syndrome. (1380988)
1992
22
Effect of corticosteroid treatment on hemopoiesis in vivo and in vitro in a patient with Felty's syndrome. (1786345)
1991
23
Ectopic splenomegaly in Felty's syndrome. (2032427)
1991
24
Worsening of Felty's syndrome with methotrexate. (1929594)
1991
25
Is there a place for splenectomy in the treatment of Felty's syndrome? (2306569)
1990
26
Resolution of Felty's syndrome with cyclophosphamide. (2754676)
1989
27
Felty's syndrome: a therapeutic dilemma? (2769662)
1989
28
Neutropenia and infections in Felty's syndrome. (3378123)
1988
29
Felty's syndrome in black Americans: a case report. (3594967)
1987
30
Simultaneous presentation of rheumatoid arthritis and acute myeloid leukaemia masquerading as Felty's syndrome. (3465419)
1986
31
Radiocolloid scintigraphy in Felty's syndrome. (3487213)
1986
32
Treatment of Felty's syndrome with low-dose oral methotrexate. (3741502)
1986
33
HLA-DR4 in Felty's syndrome. (3876440)
1985
34
Felty's syndrome in an Asian. (4016489)
1985
35
D-penicillamine in Felty's syndrome. (4057191)
1985
36
Felty's syndrome in a child. (6520839)
1984
37
Kingella kingae causing septic arthritis in Felty's syndrome. (6622347)
1983
38
High-dose immunoglobulin in Felty's syndrome. (6882047)
1983
39
HLA-DRw4 in Felty's syndrome. (6934775)
1980
40
Felty's syndrome: granulocyte-bound immunoglobulin G and splenectomy. (970768)
1976
41
Letter: Lithium in Felty's syndrome. (1259276)
1976
42
Familial Felty's syndrome. (984910)
1976
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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Search GEO for disease gene expression data for Felty's Syndrome.

Pathways for genes affiliated with Felty's Syndrome

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GO Terms for genes affiliated with Felty's Syndrome

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

idNameGO IDScoreTop Affiliating Genes
1secretory granuleGO:00301419.7ELANE, LTF
2extracellular spaceGO:00056156.7C4A, CSF2, CSF3, CXCL8, ELANE, IL3
3extracellular regionGO:00055766.4C4A, CSF2, CSF3, CXCL8, ELANE, IL3

Biological processes related to Felty's Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1positive regulation of tyrosine phosphorylation of Stat5 proteinGO:004252310.0CSF2, IL3
2positive regulation of DNA replicationGO:00457409.5CSF2, IL3
3cellular response to lipopolysaccharideGO:00712228.8CSF2, CSF3, CXCL8
4immune responseGO:00069558.0CSF2, CSF3, CXCL8, IL3

Molecular functions related to Felty's Syndrome according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1cytokine activityGO:00051259.1CSF2, CSF3, IL3
2growth factor activityGO:00080839.1CSF2, CSF3, IL3

Sources for Felty's Syndrome

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2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet