MCID: FLT011
MIFTS: 46

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, 30ICD10, 31ICD10 via Orphanet, 32ICD9CM, 39MeSH, 40MESH via Orphanet, 45NCIt, 48NIH Rare Diseases, 50Novoseek, 52OMIM, 54Orphanet, 62SNOMED-CT, 64The Human Phenotype Ontology, 68UMLS, 69UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Felty Syndrome:

Name: Felty Syndrome 52 11 48 54 39 68
Felty's Syndrome 11 48 13
Rheumatoid Arthritis with Splenoadenomegaly and Leukopenia 11
Splenomegaly-Neutropenia-Rheumatoid Arthritis Syndrome 54
 
Rheumatoid Arthritis, Splenomegaly and Neutropenia 48
Familial Felty's Syndrome 48
Feltys Syndrome 50

Characteristics:

Orphanet epidemiological data:

54
felty syndrome:
Age of onset: Adult; Age of death: elderly

HPO:

64
felty syndrome:
Inheritance: autosomal dominant inheritance

Classifications:



External Ids:

OMIM52 134750
Disease Ontology11 DOID:11042
ICD1030 M05.0, M05.00
ICD9CM32 714.1
MeSH39 D005258
SNOMED-CT62 156482001, 57160007
NCIt45 C84712
Orphanet54 ORPHA47612
MESH via Orphanet40 D005258
ICD10 via Orphanet31 M05.0
UMLS via Orphanet69 C0015773

Summaries for Felty Syndrome

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NIH Rare Diseases:48 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 abca12-related autosomal recessive congenital ichthyosis and cardiomyopathy diabetes deafness, and has symptoms including Array, Array and Array. An important gene associated with Felty Syndrome is C4A (Complement C4A (Rodgers Blood Group)), and among its related pathways are Pertussis and Malaria. Affiliated tissues include skin, liver and spleen.

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

Wikipedia:71 Felty\'s syndrome, also called Felty syndrome, is characterized by the combination of rheumatoid... more...

Description from OMIM:52 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 73)
idRelated DiseaseScoreTop Affiliating Genes
1abca12-related autosomal recessive congenital ichthyosis10.2CSF3, ELANE
2cardiomyopathy diabetes deafness10.1CXCL8, ELANE
3infectious anterior uveitis10.1CXCL8, ELANE
4omsk hemorrhagic fever10.1CXCL8, ELANE
5calpainopathy10.1CXCL8, ELANE
6adnp syndrome10.1CXCL8, ELANE
7retinitis pigmentosa 5110.1CXCL8, ELANE
8coronary arterial fistulas10.1CXCL8, LTF
9psychotic disorder10.1CXCL8, LTF
10retinal vascular disease10.1CXCL8, LTF
11infancy electroclinical syndrome10.1CXCL8, ELANE
12handl syndrome10.1CXCL8, LTF
13eosinophilic cystitis10.1CSF2, CSF3
14aniseikonia10.1CSF2, CSF3
15sclerosing cholangitis10.0CXCL8, LTF
16tracheitis10.0CXCL8, IL3
17choledochal cyst10.0CSF2, IL3
18peripheral nervous system neoplasm10.0CSF2, CSF3
19entropion10.0CXCL8, LTF
20rectum squamous cell carcinoma10.0CSF3, CXCL8, ELANE
21pharc syndrome10.0CSF2, CSF3
22cytokine receptor deficiency10.0CSF2, IL3
23alcoholic hepatitis10.0C4A, CXCL8, ELANE
24bleeding disorder, platelet-type, 1810.0CSF3, ELANE, IL3
25retinal drusen10.0CSF2, ELANE
26necrotizing gastritis10.0CSF2, IL3
27acute panmyelosis with myelofibrosis10.0CSF2, CXCL8
28esophagus lymphoma10.0CSF2, CXCL8
29colon adenocarcinoma10.0CXCL8, IL3
30malignant neoplasm of acoustic nerve10.0CXCL8, ELANE
31hidradenitis10.0CSF2, CXCL8
32blood coagulation disease10.0CSF2, CSF3
33childhood malignant mesenchymoma10.0CSF2, IL3
34paragonimiasis10.0CXCL8, LTF
35middle cerebral artery infarction10.0CSF2, IL3
36stenosis of lacrimal sac9.9CXCL8, ELANE, LTF
37granulomatous hepatitis9.9CSF2, IL3
38arthritis9.9
39grix blankenship peterson syndrome9.9CSF2, CSF3, IL3
40dic in newborn9.9CSF2, CSF3, IL3
41actinobacillosis9.9CSF2, CSF3, IL3
42inflammatory bowel disease 29.9CSF2, ELANE
43fasciitis9.9CXCL8, ELANE
44carnitine-acylcarnitine translocase deficiency9.9CSF2, CSF3, IL3
45labyrinthine bilateral reactive loss9.9C4A, CSF2, CXCL8
46peroxisome biogenesis disorder 7a9.9CSF2, CSF3, IL3
47hemophagocytic lymphohistiocytosis9.9CSF2, CSF3, IL3
48hyperparathyroidism, neonatal9.8CSF2, CSF3, IL3
49spontaneous ocular nystagmus9.8CSF2, CXCL8, ELANE
50acute chest syndrome9.8CSF2, CXCL8, ELANE

Comorbidity relations with Felty Syndrome via Phenotypic Disease Network (PDN):


Rheumatoid ArthritisDeficiency Anemia
NeutropeniaHeart Disease
Acute Cystitis

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



Diseases related to felty syndrome

Symptoms & Phenotypes 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:

 54 64 (show all 37)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 recurrent urinary tract infections64 54 Occasional (29-5%) HP:0000010
2 sinusitis64 54 Frequent (79-30%) HP:0000246
3 chronic otitis media64 54 Frequent (79-30%) HP:0000389
4 abnormal joint morphology54 Very frequent (99-80%)
5 arthritis64 54 Very frequent (99-80%) HP:0001369
6 limitation of joint mobility64 54 Very frequent (99-80%) HP:0001376
7 subcutaneous nodule64 54 Very frequent (99-80%) HP:0001482
8 pericarditis64 54 Occasional (29-5%) HP:0001701
9 splenomegaly64 54 Frequent (79-30%) HP:0001744
10 weight loss64 54 Frequent (79-30%) HP:0001824
11 thrombocytopenia64 54 Occasional (29-5%) HP:0001873
12 neutropenia64 54 Very frequent (99-80%) HP:0001875
13 anemia64 54 Frequent (79-30%) HP:0001903
14 pleuritis64 54 Occasional (29-5%) HP:0002102
15 recurrent respiratory infections54 Very frequent (99-80%)
16 pulmonary fibrosis64 54 Occasional (29-5%) HP:0002206
17 hepatomegaly64 54 Occasional (29-5%) HP:0002240
18 lymphoma64 54 Occasional (29-5%) HP:0002665
19 lymphadenopathy64 54 Frequent (79-30%) HP:0002716
20 recurrent infections54 Very frequent (99-80%)
21 immunodeficiency64 54 Very frequent (99-80%) HP:0002721
22 osteolysis64 54 Very frequent (99-80%) HP:0002797
23 arthralgia64 54 Very frequent (99-80%) HP:0002829
24 autoimmunity64 54 Very frequent (99-80%) HP:0002960
25 abnormality of lymphocytes64 54 Frequent (79-30%) HP:0004332
26 bone marrow hypocellularity64 54 Occasional (29-5%) HP:0005528
27 recurrent pneumonia64 54 Frequent (79-30%) HP:0006532
28 irregular hyperpigmentation64 54 Occasional (29-5%) HP:0007400
29 generalized hyperpigmentation64 54 Occasional (29-5%) HP:0007440
30 peripheral neuropathy64 54 Occasional (29-5%) HP:0009830
31 rhinitis64 54 Frequent (79-30%) HP:0012384
32 episcleritis64 54 Occasional (29-5%) HP:0100534
33 cellulitis64 54 Occasional (29-5%) HP:0100658
34 synovitis64 54 Very frequent (99-80%) HP:0100769
35 recurrent pharyngitis64 54 Frequent (79-30%) HP:0100776
36 sepsis64 54 Occasional (29-5%) HP:0100806
37 rheumatoid arthritis64 HP:0001370

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 2293550-18-0, 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 21220135968-09-1
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 213086
4Antirheumatic AgentsPhase 210956
5Antineoplastic Agents, AlkylatingPhase 24603
6Alkylating AgentsPhase 24827

Interventional clinical trials:

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

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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:

36
Skin, Liver, Spleen, Lymph node, Bone, Bone marrow, B cells

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
Successful treatment of a large cutaneous ulcer and improvement in the hematologic manifestations of felty syndrome with rituximab. (25417682)
2014
4
Presentation of three cases followed up with a diagnosis of Felty syndrome. (27708892)
2014
5
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
6
Fatal Staphylococcus aureus bacteremia in the Felty syndrome: a maltreatment-suspected case. (22560926)
2012
7
Histoplasmosis mimicking felty syndrome. (22832303)
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
Beneficial response to rituximab in a patient with haemolysis and refractory Felty syndrome. (18474659)
2008
10
Pseudomonas aeruginosa eyelid necrosis associated with Felty syndrome. (18645441)
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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Pathways related to Felty Syndrome according to GeneCards Suite gene sharing:

(show all 19)
idSuper pathwaysScoreTop Affiliating Genes
19.5C4A, CXCL8
29.4CSF3, CXCL8
3
Show member pathways
9.4CSF2, CXCL8
49.4CSF2, CXCL8
59.4CSF2, CXCL8
69.1CSF2, CSF3, IL3
7
Show member pathways
9.1CSF2, CSF3, IL3
89.1CSF2, CSF3, IL3
99.1CSF2, CSF3, IL3
109.1CSF2, CSF3, IL3
11
Show member pathways
9.0CSF2, CSF3, CXCL8
12
Show member pathways
9.0CSF2, CSF3, CXCL8
139.0CSF2, CSF3, CXCL8
149.0CSF2, CSF3, CXCL8
15
Show member pathways
8.9CSF2, CXCL8, IL3
16
Show member pathways
8.5CSF2, CSF3, CXCL8, IL3
178.5CSF2, CSF3, CXCL8, IL3
188.4CSF2, CXCL8, ELANE, IL3
19
Show member pathways
6.7C4A, CSF2, CSF3, CXCL8, ELANE, IL3

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
1specific granule lumenGO:00355809.1ELANE, LTF
2extracellular regionGO:00055767.5C4A, CSF2, CSF3, CXCL8, ELANE, IL3
3extracellular spaceGO:00056157.2C4A, CSF2, CSF3, CXCL8, ELANE, IL3

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

idNameGO IDScoreTop Affiliating Genes
1positive regulation of DNA replicationGO:004574010.0CSF2, IL3
2positive regulation of tyrosine phosphorylation of STAT proteinGO:00425319.8CSF2, IL3
3antimicrobial humoral responseGO:00197309.8ELANE, LTF
4cellular response to lipopolysaccharideGO:00712229.7CSF2, CSF3, CXCL8
5positive regulation of tyrosine phosphorylation of Stat5 proteinGO:00425239.5CSF2, IL3
6immune responseGO:00069559.0CSF2, CSF3, CXCL8, IL3

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

idNameGO IDScoreTop Affiliating Genes
1growth factor activityGO:00080839.7CSF2, CSF3, IL3
2cytokine activityGO:00051259.1CSF2, CSF3, CXCL8, IL3
3serine-type endopeptidase activityGO:00042528.8C4A, ELANE, LTF

Sources for Felty Syndrome

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet