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MCID: FLT011
MIFTS: 51

Felty Syndrome

Categories: Rare diseases, Bone diseases, Blood diseases, Genetic diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Felty Syndrome

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MalaCards integrated aliases for Felty Syndrome:

Name: Felty Syndrome 57 12 53 59 44 73
Felty's Syndrome 12 76 53 15
Rheumatoid Arthritis with Splenoadenomegaly and Leukopenia 12
Splenomegaly-Neutropenia-Rheumatoid Arthritis Syndrome 59
Rheumatoid Arthritis, Splenomegaly and Neutropenia 53
Familial Felty's Syndrome 53
Feltys Syndrome 55

Characteristics:

Orphanet epidemiological data:

59
felty syndrome
Age of onset: Adult; Age of death: elderly;

OMIM:

57
Inheritance:
autosomal dominant


HPO:

32
felty syndrome:
Inheritance autosomal dominant inheritance


Classifications:

MalaCards categories:
Global: Rare diseases Genetic diseases
Anatomical: Bone diseases Blood diseases
See all MalaCards categories (disease lists)
ICD10: 33 34
Orphanet: 59  
Rare systemic and rhumatological diseases
Rare immunological diseases


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Summaries for Felty Syndrome

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NIH Rare Diseases : 53 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.

MalaCards based summary : Felty Syndrome, also known as felty's syndrome, is related to neutropenia and nodular regenerative hyperplasia. An important gene associated with Felty Syndrome is C4A (Complement C4A (Rodgers Blood Group)), and among its related pathways/superpathways are Innate Immune System and PEDF Induced Signaling. The drugs Cyclophosphamide and Lenograstim have been mentioned in the context of this disorder. Affiliated tissues include skin, liver and lymph node, and related phenotypes are chronic otitis media and arthritis

Disease Ontology : 12 A syndrome that results in rheumatoid arthritis, splenomegaly and neutropenia.

Wikipedia : 76 Felty\'s syndrome, also called Felty syndrome, (FS) is rare autoimmune disease characterized by the... more...

Description from OMIM: 134750
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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 72)
# Related Disease Score Top Affiliating Genes
1 neutropenia 28.0 CSF2 CSF3 ELANE IL3
2 nodular regenerative hyperplasia 11.0
3 zygomycosis 10.5 CSF3 ELANE
4 cardiac rupture 10.3 CXCL8 ELANE
5 trichosporonosis 10.3 CSF2 CSF3
6 status asthmaticus 10.3 CXCL8 ELANE
7 periodontosis 10.2 CXCL8 ELANE
8 ecthyma 10.2 CSF2 CSF3
9 alpha-1-antitrypsin deficiency 10.2 CXCL8 ELANE
10 bronchopulmonary dysplasia 10.2 CXCL8 ELANE
11 acute respiratory distress syndrome 10.2 CXCL8 ELANE
12 chorioamnionitis 10.2 CXCL8 ELANE
13 gingivitis 10.1 CXCL8 ELANE
14 mucositis 10.1 CSF2 CSF3
15 autoinflammation, lipodystrophy, and dermatosis syndrome 10.1 CXCL8 ELANE
16 acute monoblastic leukemia 10.1 CSF2 CXCL8
17 meconium aspiration syndrome 10.1 CSF2 CXCL8
18 pharyngitis 10.0 CSF2 CXCL8
19 idiopathic interstitial pneumonia 10.0 CXCL8 ELANE
20 bronchiectasis 10.0 CXCL8 ELANE
21 arthritis 10.0
22 bone inflammation disease 10.0 CXCL8 TNFRSF10A
23 bronchitis 10.0 CXCL8 ELANE
24 mastitis 10.0 CSF2 CXCL8
25 rheumatoid arthritis 10.0
26 stomatitis 9.9 CSF2 CSF3
27 leukemia 9.9
28 large granular lymphocyte leukemia 9.9
29 pneumonia 9.9 CSF3 CXCL8 ELANE
30 acute lymphocytic leukemia 9.8 CSF2 CSF3
31 adult respiratory distress syndrome 9.8 C4A CXCL8 ELANE
32 juvenile rheumatoid arthritis 9.8
33 skeletal tuberculosis 9.8 IL3 TNFRSF10A
34 seborrheic infantile dermatitis 9.8 CSF2 IL3
35 keratoconjunctivitis 9.7 CXCL8 IL3
36 eosinophilic gastroenteritis 9.7 CSF2 IL3
37 monocytic leukemia 9.7 CSF2 CXCL8
38 engraftment syndrome 9.7 CSF2 CSF3 CXCL8
39 eosinophilic pneumonia 9.7 CSF2 IL3
40 spondyloarthropathy 1 9.7
41 psoriatic arthritis 9.7
42 thrombocytopenia 9.7
43 rheumatic disease 9.7
44 histoplasmosis 9.7
45 enthesopathy 9.7
46 spondylitis 9.7
47 acute pancreatitis 9.7 CXCL8 ELANE
48 myeloma, multiple 9.6 CSF2 CSF3 CXCL8
49 lymphoma, hodgkin, classic 9.6 CSF2 IL3
50 bronchial disease 9.6 CSF2 CXCL8 ELANE

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


Acute Cystitis Deficiency Anemia
Heart Disease Neutropenia
Rheumatoid Arthritis

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



Diseases related to Felty Syndrome
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Symptoms & Phenotypes for Felty Syndrome

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Symptoms via clinical synopsis from OMIM:

57
GI:
splenomegaly

Joints:
rheumatoid arthritis

Heme:
neutropenia

Misc:
confined mainly to white males
hla-drw4 association


Clinical features from OMIM:

134750

Human phenotypes related to Felty Syndrome:

59 32 (show all 38)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 chronic otitis media 59 32 frequent (33%) Frequent (79-30%) HP:0000389
2 arthritis 59 32 hallmark (90%) Very frequent (99-80%) HP:0001369
3 splenomegaly 59 32 frequent (33%) Frequent (79-30%) HP:0001744
4 hepatomegaly 59 32 occasional (7.5%) Occasional (29-5%) HP:0002240
5 subcutaneous nodule 59 32 hallmark (90%) Very frequent (99-80%) HP:0001482
6 pulmonary fibrosis 59 32 occasional (7.5%) Occasional (29-5%) HP:0002206
7 arthralgia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002829
8 immunodeficiency 59 32 hallmark (90%) Very frequent (99-80%) HP:0002721
9 anemia 59 32 frequent (33%) Frequent (79-30%) HP:0001903
10 bone marrow hypocellularity 59 32 occasional (7.5%) Occasional (29-5%) HP:0005528
11 weight loss 59 32 frequent (33%) Frequent (79-30%) HP:0001824
12 peripheral neuropathy 59 32 occasional (7.5%) Occasional (29-5%) HP:0009830
13 sinusitis 59 32 frequent (33%) Frequent (79-30%) HP:0000246
14 limitation of joint mobility 59 32 hallmark (90%) Very frequent (99-80%) HP:0001376
15 autoimmunity 59 32 hallmark (90%) Very frequent (99-80%) HP:0002960
16 thrombocytopenia 59 32 occasional (7.5%) Occasional (29-5%) HP:0001873
17 irregular hyperpigmentation 59 32 occasional (7.5%) Occasional (29-5%) HP:0007400
18 pericarditis 59 32 occasional (7.5%) Occasional (29-5%) HP:0001701
19 recurrent pharyngitis 59 32 frequent (33%) Frequent (79-30%) HP:0100776
20 neutropenia 59 32 hallmark (90%) Very frequent (99-80%) HP:0001875
21 recurrent pneumonia 59 32 frequent (33%) Frequent (79-30%) HP:0006532
22 cellulitis 59 32 occasional (7.5%) Occasional (29-5%) HP:0100658
23 sepsis 59 32 occasional (7.5%) Occasional (29-5%) HP:0100806
24 lymphoma 59 32 occasional (7.5%) Occasional (29-5%) HP:0002665
25 recurrent urinary tract infections 59 32 occasional (7.5%) Occasional (29-5%) HP:0000010
26 osteolysis 59 32 hallmark (90%) Very frequent (99-80%) HP:0002797
27 lymphadenopathy 59 32 frequent (33%) Frequent (79-30%) HP:0002716
28 generalized hyperpigmentation 59 32 occasional (7.5%) Occasional (29-5%) HP:0007440
29 pleuritis 59 32 occasional (7.5%) Occasional (29-5%) HP:0002102
30 episcleritis 59 32 occasional (7.5%) Occasional (29-5%) HP:0100534
31 rhinitis 59 32 frequent (33%) Frequent (79-30%) HP:0012384
32 synovitis 59 32 hallmark (90%) Very frequent (99-80%) HP:0100769
33 recurrent respiratory infections 59 Very frequent (99-80%)
34 recurrent infections 59 Very frequent (99-80%)
35 rheumatoid arthritis 32 HP:0001370
36 abnormal joint morphology 59 Very frequent (99-80%)
37 abnormality of lymphocytes 59 Frequent (79-30%)
38 abnormal lymphocyte morphology 32 frequent (33%) HP:0004332
Sources

Drugs & Therapeutics for Felty Syndrome

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

(show all 6)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
2
Lenograstim Approved, Investigational Phase 2 135968-09-1
3 Alkylating Agents Phase 2
4 Antineoplastic Agents, Alkylating Phase 2
5 Antirheumatic Agents Phase 2
6 Immunosuppressive Agents Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Phase II Study of High-Dose Cyclophosphamide in Patients With Severe Autoimmune Hematologic Disease Completed NCT00010387 Phase 2 cyclophosphamide;filgrastim
2 An Observational Study to Describe in Routine Clinical Practice the Treatment Patterns of Usage of Biological DMARDs in RA Patients. Completed NCT02590562
3 An Observational Study in Clinical Practice Management of Patients With Biological Drugs in Monotherapy Completed NCT01664117

Search NIH Clinical Center for Felty Syndrome

Cochrane evidence based reviews: felty syndrome

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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:

41
Skin, Liver, Lymph Node, Spleen, Bone, Bone Marrow, B Cells
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Publications for Felty Syndrome

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Articles related to Felty Syndrome:

(show all 24)
# Title Authors Year
1
Somatic<i>STAT3</i>mutations in Felty syndrome: an implication for a common pathogenesis with large granular lymphocyte leukemia. ( 29217783 )
2018
2
&amp;quot;Rituximab: A Safe Treatment in a Patient With Refractory Felty Syndrome and Recurrent Infections&amp;quot;. ( 28002164 )
2017
3
A case of rheumatoid arthritis with Felty syndrome complicated by late-onset ankylosing spondylitis and psoriatic arthritis: a potpourri of rheumatic diseases. ( 25539441 )
2015
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
Presentation of three cases followed up with a diagnosis of Felty syndrome. ( 27708892 )
2014
6
Successful treatment of a large cutaneous ulcer and improvement in the hematologic manifestations of felty syndrome with rituximab. ( 25417682 )
2014
7
Histoplasmosis mimicking felty syndrome. ( 22832303 )
2012
8
Fatal Staphylococcus aureus bacteremia in the Felty syndrome: a maltreatment-suspected case. ( 22560926 )
2012
9
Leg ulcer and thigh telangiectasia associated with natural killer cell CD56(-) large granular lymphocyte leukemia in a patient with pseudo-Felty syndrome. ( 19962215 )
2010
10
Beneficial response to rituximab in a patient with haemolysis and refractory Felty syndrome. ( 18474659 )
2008
11
Pseudomonas aeruginosa eyelid necrosis associated with Felty syndrome. ( 18645441 )
2008
12
Beneficial response to rituximab in refractory Felty Syndrome. ( 16484886 )
2006
13
Pathogenesis of neutropenia in large granular lymphocyte leukemia and Felty syndrome. ( 16530306 )
2006
14
A case of adult-onset Felty syndrome in juvenile rheumatoid arthritis. ( 16118895 )
2005
15
Felty syndrome complicating juvenile rheumatoid arthritis. ( 9787332 )
1998
16
An unusual association of Felty syndrome and TCR gamma delta lymphocytosis. ( 8655718 )
1996
17
[Felty syndrome: a therapy-resistant variant of chronic rheumatoid arthritis? 2 case reports and literature review]. ( 8657998 )
1996
18
Treatment of the neutropenia of Felty syndrome. ( 8932830 )
1996
19
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
20
Incidence of cancer among men with the Felty syndrome. ( 8250454 )
1994
21
The Felty syndrome and G-CSF-associated thrombocytopenia and severe anemia. ( 7678374 )
1993
22
GM-CSF in the treatment of Felty syndrome. ( 2024642 )
1991
23
The Felty syndrome: a case-matched study of clinical manifestations and outcome, serologic features, and immunogenetic associations. ( 1969604 )
1990
24
Parenteral gold therapy in the Felty syndrome. Experience with 20 patients. ( 3951357 )
1986
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Variations for Felty Syndrome

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Expression for Felty Syndrome

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Search GEO for disease gene expression data for Felty Syndrome.
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Pathways for Felty Syndrome

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Pathways related to Felty Syndrome according to GeneCards Suite gene sharing:

(show all 25)
# Super pathways Score Top Affiliating Genes
1
Innate Immune System 66
Show member pathways
 13.64 C4A CSF2 CSF3 CXCL8 ELANE IL3
2
PEDF Induced Signaling 64
Show member pathways
 13.31 CSF2 CSF3 CXCL8 IL3 TNFRSF10A
3
Akt Signaling 64
Show member pathways
 13.05 CSF2 CSF3 CXCL8 IL3 TNFRSF10A
4
Cytokine Signaling in Immune system 66
Show member pathways
 13.02 CSF2 CSF3 CXCL8 IL3
5
PAK Pathway 64
Show member pathways
 12.96 CSF2 CSF3 CXCL8 IL3 TNFRSF10A
6
Regulation of IFNA signaling 66
Show member pathways
 12.26 CSF2 CSF3 CXCL8 IL3
7
Immune response Role of DAP12 receptors in NK cells 22
Show member pathways
 12.2 CSF2 IL3 TNFRSF10A
8
IL-17 Family Signaling Pathways 65
Show member pathways
 12.03 CSF2 CSF3 CXCL8
9
Immune response IFN gamma signaling pathway 22
Show member pathways
 11.77 CXCL8 IL3 TNFRSF10A
10
Hematopoietic Stem Cell Differentiation Pathways and Lineage-specific Markers 65
11.72 CSF2 CSF3 IL3
11
Hematopoietic cell lineage 37
11.62 CSF2 CSF3 IL3
12
Rheumatoid arthritis 37
11.58 CSF2 CXCL8
13
Cytokine production by Th17 cells in CF (Mouse model) 22
Show member pathways
 11.58 CSF2 CSF3 CXCL8
14
Salmonella infection (KEGG) 37
11.55 CSF2 CXCL8
15
Glucocorticoid receptor regulatory network 1
11.53 CSF2 CXCL8
16
Transcriptional misregulation in cancer 37
11.53 CSF2 CXCL8 ELANE IL3
17
Pertussis 37
11.48 C4A CXCL8
18
Calcineurin-regulated NFAT-dependent transcription in lymphocytes 1
Show member pathways
 11.46 CSF2 CXCL8 IL3
19
Photodynamic therapy-induced NF-kB survival signaling 1
Show member pathways
 11.39 CSF2 CXCL8
20
Lung fibrosis 1
11.3 CSF2 CSF3 CXCL8
21
Malaria 37
11.25 CSF3 CXCL8
22
Interleukin-10 signaling 66
11.12 CSF2 CSF3 CXCL8
23
Syndecan-2-mediated signaling events 1
11.05 CSF2 CXCL8
24
Hematopoietic Stem Cell Differentiation 1
11.01 CSF2 CSF3 IL3
25
Cytokines and Inflammatory Response 1
10.28 CSF2 CSF3 IL3
Sources

GO Terms for Felty Syndrome

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

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.43 C4A CSF2 CSF3 CXCL8 ELANE IL3
2 extracellular space GO:0005615 9.1 C4A CSF2 CSF3 CXCL8 ELANE IL3

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

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 inflammatory response GO:0006954 9.63 C4A CXCL8 TNFRSF10A
2 regulation of cell proliferation GO:0042127 9.58 CSF2 CXCL8 TNFRSF10A
3 regulation of signaling receptor activity GO:0010469 9.56 CSF2 CSF3 CXCL8 IL3
4 response to lipopolysaccharide GO:0032496 9.54 CXCL8 ELANE TNFRSF10A
5 cellular response to lipopolysaccharide GO:0071222 9.5 CSF2 CSF3 CXCL8
6 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.49 CSF3 IL3
7 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.48 CSF2 IL3
8 cytokine-mediated signaling pathway GO:0019221 9.46 CSF2 CSF3 CXCL8 IL3
9 positive regulation of DNA replication GO:0045740 9.43 CSF2 IL3
10 immune response GO:0006955 9.02 CSF2 CSF3 CXCL8 IL3 TNFRSF10A
11 neutrophil differentiation GO:0030223 8.96 CSF2 CSF3

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

# Name GO ID Score Top Affiliating Genes
1 growth factor activity GO:0008083 9.13 CSF2 CSF3 IL3
2 cytokine activity GO:0005125 8.92 CSF2 CSF3 CXCL8 IL3
Sources

Sources for Felty Syndrome

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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