MCID: FLT011
MIFTS: 45

Felty Syndrome malady

Categories: Genetic diseases, Rare diseases, Bone diseases, Blood diseases

Aliases & Classifications for Felty Syndrome

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Sources:
11Disease Ontology, 13DISEASES, 29ICD10, 30ICD10 via Orphanet, 31ICD9CM, 38MeSH, 39MESH via Orphanet, 44NCIt, 47NIH Rare Diseases, 49Novoseek, 51OMIM, 53Orphanet, 61SNOMED-CT, 63The Human Phenotype Ontology, 67UMLS, 68UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Felty Syndrome:

Name: Felty Syndrome 51 11 47 53 38 67
Felty's Syndrome 11 47 13
Rheumatoid Arthritis with Splenoadenomegaly and Leukopenia 11
Splenomegaly-Neutropenia-Rheumatoid Arthritis Syndrome 53
 
Rheumatoid Arthritis, Splenomegaly and Neutropenia 47
Familial Felty's Syndrome 47
Feltys Syndrome 49

Characteristics:

Orphanet epidemiological data:

53
felty syndrome:
Age of onset: Adult; Age of death: elderly

HPO:

63
felty syndrome:
Inheritance: autosomal dominant inheritance

Classifications:



External Ids:

OMIM51 134750
Disease Ontology11 DOID:11042
ICD1029 M05.0, M05.00
ICD9CM31 714.1
MeSH38 D005258
SNOMED-CT61 156482001, 57160007
NCIt44 C84712
Orphanet53 ORPHA47612
MESH via Orphanet39 D005258
ICD10 via Orphanet30 M05.0
UMLS via Orphanet68 C0015773

Summaries for Felty Syndrome

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NIH Rare Diseases:47 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: 2/21/2016

MalaCards based summary: Felty Syndrome, also known as felty's syndrome, is related to autosomal dominant cerebellar ataxia and 46,xx gonadal dysgenesis epibulbar dermoid, and has symptoms including arthritis, limitation of joint mobility and abnormality of neutrophils. An important gene associated with Felty Syndrome is C4A (Complement C4A (Rodgers Blood Group)), and among its related pathways are Cytokines and Inflammatory Response and Pertussis. Affiliated tissues include skin, liver and lymph node.

Disease Ontology:11 A syndrome that results in rheumatoid arthritis, splenomegaly and neutropenia.

Wikipedia:70 Felty\'s syndrome, also called Felty syndrome, is characterized by the combination of rheumatoid... more...

Description from OMIM:51 134750

Related Diseases for Felty Syndrome

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

(show top 50)    (show all 69)
idRelated DiseaseScoreTop Affiliating Genes
1autosomal dominant cerebellar ataxia10.2C4A, CXCL8
246,xx gonadal dysgenesis epibulbar dermoid10.2CSF3, ELANE
3platelet storage pool deficiency10.2CSF3, IL3
43-alpha hydroxyacyl-coa dehydrogenase deficiency10.2CXCL8, LTF
5pseudoretinitis pigmentosa10.2CXCL8, LTF
6spastic entropion10.1CXCL8, LTF
7cardiocranial syndrome10.1CXCL8, ELANE
8orbital plasma cell granuloma10.1CXCL8, ELANE
9chronic endophthalmitis10.1CXCL8, LTF
10enteropathica10.0CSF2, CSF3
11marchiafava bignami disease10.0CXCL8, ELANE
12meibomian cyst10.0CXCL8, ELANE
13primary systemic mycosis10.0CSF2, CSF3
14exophthalmos10.0CXCL8, IL3
15bardet-biedl syndrome 810.0CXCL8, ELANE
16neonatal period electroclinical syndrome10.0CXCL8, ELANE
17tinea cruris10.0CSF2, CSF3
18nervous system cancer10.0CXCL8, ELANE
19lymphopenia9.9CXCL8, ELANE
20severe nonproliferative diabetic retinopathy9.9CSF2, IL3
21cyp2c19-related poor drug metabolism9.9CSF2, IL3
22neurotic disorder9.9CSF2, CSF3
23benign adult familial myoclonic epilepsy9.9CSF2, CXCL8
24acute posterior multifocal placoid pigment epitheliopathy9.9CSF2, CXCL8
25arthritis9.9
26pasteurellosis9.9CSF2, CXCL8
27subcorneal pustular dermatosis9.9CXCL8, ELANE
28microcystic adenoma9.8CSF2, IL3
29rheumatoid arthritis9.8
30neutropenia9.8
31nonspecific interstitial pneumonia9.8CXCL8, ELANE
32single ventricular heart9.8CSF3, ELANE, IL3
33basaloid squamous cell carcinoma9.8CSF3, CXCL8, ELANE
34alcohol abuse9.8C4A, CXCL8, ELANE
35herpes simplex encephalitis 19.8CSF3, ELANE, IL3
36leukemia9.7
37large granular lymphocyte leukemia9.7
38blind hypotensive eye9.7CSF2, CXCL8
39hodgkin's lymphoma, mixed cellularity9.7CXCL8, ELANE
40alternating exotropia9.7CSF2, IL3
41acute myocardial infarction9.7CSF2, CSF3
42palmoplantar keratosis9.6CXCL8, ELANE
43transient neonatal neutropenia9.6CSF2, CSF3, IL3
44hemolytic anemia9.6CSF2, CSF3, IL3
45organ system benign neoplasm9.6CSF2, CSF3, IL3
46transmitted_by9.6CSF2, IL3
47gemistocytic astrocytoma9.6CSF2, CSF3, CXCL8
48peroxisome biogenesis disorder 7a9.6CSF2, CSF3, IL3
49bacteremia9.6
50psoriatic arthritis9.6

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



Diseases related to felty syndrome

Symptoms for Felty Syndrome

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Symptoms by clinical synopsis from OMIM:

134750

Clinical features from OMIM:

134750

Human phenotypes related to Felty Syndrome:

 63 53 (show all 43)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 arthritis63 53 hallmark (90%) Very frequent (99-80%) HP:0001369
2 limitation of joint mobility63 53 hallmark (90%) Very frequent (99-80%) HP:0001376
3 abnormality of neutrophils63 hallmark (90%) HP:0001874
4 osteolysis63 53 hallmark (90%) Very frequent (99-80%) HP:0002797
5 arthralgia63 53 hallmark (90%) Very frequent (99-80%) HP:0002829
6 autoimmunity63 53 hallmark (90%) Very frequent (99-80%) HP:0002960
7 sinusitis63 53 typical (50%) Frequent (79-30%) HP:0000246
8 otitis media63 typical (50%) HP:0000388
9 splenomegaly63 53 typical (50%) Frequent (79-30%) HP:0001744
10 weight loss63 53 typical (50%) Frequent (79-30%) HP:0001824
11 anemia63 53 typical (50%) Frequent (79-30%) HP:0001903
12 lymphadenopathy63 53 typical (50%) Frequent (79-30%) HP:0002716
13 abnormality of lymphocytes63 53 typical (50%) Frequent (79-30%) HP:0004332
14 recurrent pharyngitis63 53 typical (50%) Frequent (79-30%) HP:0100776
15 recurrent urinary tract infections63 53 occasional (7.5%) Occasional (29-5%) HP:0000010
16 abnormality of the pericardium63 occasional (7.5%) HP:0001697
17 thrombocytopenia63 53 occasional (7.5%) Occasional (29-5%) HP:0001873
18 abnormality of the pleura63 occasional (7.5%) HP:0002103
19 pulmonary fibrosis63 53 occasional (7.5%) Occasional (29-5%) HP:0002206
20 hepatomegaly63 53 occasional (7.5%) Occasional (29-5%) HP:0002240
21 lymphoma63 53 occasional (7.5%) Occasional (29-5%) HP:0002665
22 bone marrow hypocellularity63 53 occasional (7.5%) Occasional (29-5%) HP:0005528
23 irregular hyperpigmentation63 53 occasional (7.5%) Occasional (29-5%) HP:0007400
24 generalized hyperpigmentation63 53 occasional (7.5%) Occasional (29-5%) HP:0007440
25 peripheral neuropathy63 53 occasional (7.5%) Occasional (29-5%) HP:0009830
26 inflammatory abnormality of the eye63 occasional (7.5%) HP:0100533
27 cellulitis63 53 occasional (7.5%) Occasional (29-5%) HP:0100658
28 sepsis63 53 occasional (7.5%) Occasional (29-5%) HP:0100806
29 skin ulcer63 occasional (7.5%) HP:0200042
30 rheumatoid arthritis63 HP:0001370
31 neutropenia63 53 Very frequent (99-80%) HP:0001875
32 chronic otitis media53 Frequent (79-30%)
33 abnormal joint morphology53 Very frequent (99-80%)
34 subcutaneous nodule53 Very frequent (99-80%)
35 pericarditis53 Occasional (29-5%)
36 pleuritis53 Occasional (29-5%)
37 recurrent respiratory infections53 Very frequent (99-80%)
38 recurrent infections53 Very frequent (99-80%)
39 immunodeficiency53 Very frequent (99-80%)
40 recurrent pneumonia53 Frequent (79-30%)
41 rhinitis53 Frequent (79-30%)
42 episcleritis53 Occasional (29-5%)
43 synovitis53 Very frequent (99-80%)

Drugs & Therapeutics for Felty Syndrome

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

(show all 6)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
Cyclophosphamideapproved, investigationalPhase 2282950-18-0, 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
Anhydrous cyclophosphamide
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
2
LenograstimapprovedPhase 21202135968-09-1
Synonyms:
G-CSF (CHO cell derived)
Glycosylated recombinant G-CSF
Glycosylated recombinant granulocyte colony stimulating factor
 
Granulocyte colony stimulating factor 3 (CHO cell derived)
Granulocyte colony-stimulating factor lenograstim
Lenograstim (genetical recombination)
Lenograstim rDNA
3Immunosuppressive AgentsPhase 212770
4Antirheumatic AgentsPhase 210627
5Antineoplastic Agents, AlkylatingPhase 24474
6Alkylating AgentsPhase 24694

Interventional clinical trials:

idNameStatusNCT IDPhase
1Phase II Study of High-Dose Cyclophosphamide in Patients With Severe Autoimmune Hematologic DiseaseCompletedNCT00010387Phase 2

Search NIH Clinical Center for Felty Syndrome


Cochrane evidence based reviews: felty syndrome

Genetic Tests for Felty Syndrome

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Anatomical Context for Felty Syndrome

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MalaCards organs/tissues related to Felty Syndrome:

35
Skin, Liver, Lymph node, Spleen, Bone, Neutrophil, Eye

Animal Models for Felty Syndrome or affiliated genes

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Publications for Felty Syndrome

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Articles related to Felty Syndrome:

(show all 22)
idTitleAuthorsYear
1
Rituximab: A Safe Treatment in a Patient With Refractory Felty Syndrome and Recurrent Infections". (28002164)
2017
2
A case of rheumatoid arthritis with Felty syndrome complicated by late-onset ankylosing spondylitis and psoriatic arthritis: a potpourri of rheumatic diseases. (25539441)
2015
3
Presentation of three cases followed up with a diagnosis of Felty syndrome. (27708892)
2014
4
A beneficial long-term and consistent response to rituximab in the treatment of refractory neutropenia and arthritis in a patient with Felty syndrome. (25275774)
2014
5
Successful treatment of a large cutaneous ulcer and improvement in the hematologic manifestations of felty syndrome with rituximab. (25417682)
2014
6
Histoplasmosis mimicking felty syndrome. (22832303)
2012
7
Fatal Staphylococcus aureus bacteremia in the Felty syndrome: a maltreatment-suspected case. (22560926)
2012
8
Leg ulcer and thigh telangiectasia associated with natural killer cell CD56(-) large granular lymphocyte leukemia in a patient with pseudo-Felty syndrome. (19962215)
2010
9
Pseudomonas aeruginosa eyelid necrosis associated with Felty syndrome. (18645441)
2008
10
Beneficial response to rituximab in a patient with haemolysis and refractory Felty syndrome. (18474659)
2008
11
Pathogenesis of neutropenia in large granular lymphocyte leukemia and Felty syndrome. (16530306)
2006
12
Beneficial response to rituximab in refractory Felty Syndrome. (16484886)
2006
13
A case of adult-onset Felty syndrome in juvenile rheumatoid arthritis. (16118895)
2005
14
Felty syndrome complicating juvenile rheumatoid arthritis. (9787332)
1998
15
Treatment of the neutropenia of Felty syndrome. (8932830)
1996
16
An unusual association of Felty syndrome and TCR gamma delta lymphocytosis. (8655718)
1996
17
V gamma 9-V delta 2+ gamma delta T cells from a patient with Felty syndrome that exhibit aberrant response to triggering of the CD3 molecule can regulate immunoglobulin secretion by B cells. (7828370)
1995
18
Incidence of cancer among men with the Felty syndrome. (8250454)
1994
19
The Felty syndrome and G-CSF-associated thrombocytopenia and severe anemia. (7678374)
1993
20
GM-CSF in the treatment of Felty syndrome. (2024642)
1991
21
The Felty syndrome: a case-matched study of clinical manifestations and outcome, serologic features, and immunogenetic associations. (1969604)
1990
22
Parenteral gold therapy in the Felty syndrome. Experience with 20 patients. (3951357)
1986

Variations for Felty Syndrome

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Expression for genes affiliated with Felty Syndrome

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Search GEO for disease gene expression data for Felty Syndrome.

Pathways for genes affiliated with Felty Syndrome

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GO Terms for genes affiliated with Felty Syndrome

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Cellular components related to Felty 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 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 peptidyl-tyrosine phosphorylationGO:00507319.9CSF3, IL3
3positive regulation of DNA replicationGO:00457409.8CSF2, IL3
4cellular response to lipopolysaccharideGO:00712229.4CSF2, CSF3, CXCL8
5proteolysisGO:00065088.8C4A, ELANE, LTF
6immune responseGO:00069558.7CSF2, CSF3, CXCL8, IL3

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

idNameGO IDScoreTop Affiliating Genes
1cytokine activityGO:00051259.2CSF2, CSF3, IL3
2growth factor activityGO:00080838.8CSF2, CSF3, IL3
3serine-type endopeptidase activityGO:00042528.8C4A, ELANE, LTF

Sources for Felty Syndrome

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
26GTR
27HGMD
28HMDB
29ICD10
30ICD10 via Orphanet
31ICD9CM
32IUPHAR
33KEGG
36MedGen
38MeSH
39MESH via Orphanet
40MGI
43NCI
44NCIt
45NDF-RT
48NINDS
49Novoseek
51OMIM
52OMIM via Orphanet
56PubMed
57QIAGEN
62SNOMED-CT via Orphanet
66Tumor Gene Family of Databases
67UMLS
68UMLS via Orphanet