MCID: FLT011
MIFTS: 51

Felty Syndrome

Categories: Blood diseases, Bone diseases, Rare diseases

Aliases & Classifications for Felty Syndrome

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:



Summaries for Felty Syndrome

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 Lenograstim and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include spleen, neutrophil and liver, 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

Related Diseases for Felty Syndrome

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

(show top 50) (show all 59)
# Related Disease Score Top Affiliating Genes
1 neutropenia 29.9 IL3 CSF3 CSF2
2 nodular regenerative hyperplasia 11.1
3 arthritis 10.2
4 rheumatoid arthritis 10.2
5 pouchitis 10.1 LTF CXCL8
6 giant papillary conjunctivitis 10.1 LTF CXCL8
7 haemophilus influenzae 10.1 LTF CXCL8
8 trichosporonosis 10.1 CSF3 CSF2
9 adult respiratory distress syndrome 10.1 CXCL8 C4A
10 leukemia 10.1
11 large granular lymphocyte leukemia 10.1
12 aseptic meningitis 10.1 LTF CXCL8
13 oral candidiasis 10.1 LTF CXCL8
14 ecthyma 10.0 CSF3 CSF2
15 shigellosis 10.0 LTF CXCL8
16 aggressive periodontitis 10.0 LTF CXCL8
17 gingivitis 10.0 LTF CXCL8
18 acute monoblastic leukemia 10.0 CXCL8 CSF2
19 meconium aspiration syndrome 10.0 CXCL8 CSF2
20 mucositis 10.0 CSF3 CSF2
21 pharyngitis 10.0 CXCL8 CSF2
22 stomatitis 10.0 CSF3 CSF2
23 bone inflammation disease 10.0 TNFRSF10A CXCL8
24 juvenile rheumatoid arthritis 10.0
25 bronchial disease 9.9 CXCL8 CSF2
26 acute lymphocytic leukemia 9.9 CSF3 CSF2
27 monocytic leukemia 9.9 CXCL8 CSF2
28 seborrheic infantile dermatitis 9.9 IL3 CSF2
29 keratoconjunctivitis 9.9 IL3 CXCL8
30 engraftment syndrome 9.9 CXCL8 CSF3 CSF2
31 bacterial infectious disease 9.9 CXCL8 CSF3 CSF2
32 eosinophilic pneumonia 9.9 IL3 CSF2
33 capillary leak syndrome 9.9 CXCL8 CSF2 C4A
34 myeloma, multiple 9.9 CXCL8 CSF3 CSF2
35 mastitis 9.9 LTF CXCL8 CSF2
36 thrombocytosis 9.8 IL3 CSF2
37 spondyloarthropathy 1 9.8
38 psoriatic arthritis 9.8
39 thrombocytopenia 9.8
40 histoplasmosis 9.8
41 spondylitis 9.8
42 megakaryocytic leukemia 9.8 IL3 CSF2
43 diphtheria 9.8 IL3 CSF2
44 granulocytopenia 9.8 IL3 CSF3 CSF2
45 anemia of prematurity 9.8 IL3 CSF3 CSF2
46 leukocyte disease 9.8 IL3 CSF3 CSF2
47 pancytopenia 9.8 IL3 CSF3 CSF2
48 severe congenital neutropenia 9.8 IL3 CSF3 CSF2
49 bone marrow cancer 9.8 IL3 CSF3 CSF2
50 leukemia, chronic myeloid 9.8 IL3 CSF3 CSF2

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

Symptoms & Phenotypes for Felty Syndrome

Symptoms via clinical synopsis from OMIM:

57
G I:
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

Drugs & Therapeutics for Felty Syndrome

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

(show all 7)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Lenograstim Approved, Investigational Phase 2 135968-09-1
2
Cyclophosphamide Approved, Investigational Phase 2 6055-19-2, 50-18-0 2907
3 Immunosuppressive Agents Phase 2
4 Immunologic Factors Phase 2
5 Antineoplastic Agents, Alkylating Phase 2
6 Alkylating Agents Phase 2
7 Antirheumatic 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

Genetic Tests for Felty Syndrome

Anatomical Context for Felty Syndrome

MalaCards organs/tissues related to Felty Syndrome:

41
Spleen, Neutrophil, Liver, Skin, Lymph Node, Bone, Bone Marrow

Publications for Felty Syndrome

Articles related to Felty Syndrome:

(show top 50) (show all 60)
# 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
A Case Report of Chikungunya Fever, Rheumatoid Arthritis, and Felty's Syndrome. ( 29536378 )
2018
3
Successful treatment of refractory neutropenia in Felty's syndrome with rituximab. ( 28753121 )
2018
4
&amp;quot;Rituximab: A Safe Treatment in a Patient With Refractory Felty Syndrome and Recurrent Infections&amp;quot;. ( 28002164 )
2017
5
Felty's Syndrome: A Qualitative Case Study. ( 30304590 )
2017
6
Primary cardiac B cell lymphoma: Manifestation of Felty's syndrome or TNFα antagonist. ( 27773517 )
2016
7
A case of rheumatoid arthritis with Felty syndrome complicated by late-onset ankylosing spondylitis and psoriatic arthritis: a potpourri of rheumatic diseases. ( 25539441 )
2015
8
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
9
Presentation of three cases followed up with a diagnosis of Felty syndrome. ( 27708892 )
2014
10
Successful treatment of a large cutaneous ulcer and improvement in the hematologic manifestations of felty syndrome with rituximab. ( 25417682 )
2014
11
Felty's syndrome with hyperthyroidism: a case report and literature review. ( 24472276 )
2014
12
Histoplasmosis mimicking felty syndrome. ( 22832303 )
2012
13
Fatal Staphylococcus aureus bacteremia in the Felty syndrome: a maltreatment-suspected case. ( 22560926 )
2012
14
Leg ulcer and thigh telangiectasia associated with natural killer cell CD56(-) large granular lymphocyte leukemia in a patient with pseudo-Felty syndrome. ( 19962215 )
2010
15
Beneficial response to rituximab in a patient with haemolysis and refractory Felty syndrome. ( 18474659 )
2008
16
Pseudomonas aeruginosa eyelid necrosis associated with Felty syndrome. ( 18645441 )
2008
17
Beneficial response to rituximab in refractory Felty Syndrome. ( 16484886 )
2006
18
Pathogenesis of neutropenia in large granular lymphocyte leukemia and Felty syndrome. ( 16530306 )
2006
19
A case of adult-onset Felty syndrome in juvenile rheumatoid arthritis. ( 16118895 )
2005
20
Hyperviscosity syndrome in rheumatoid arthritis with Felty's syndrome: case report and review of the literature. ( 11954894 )
2002
21
Low Fcgamma receptor III and high granulocyte colony-stimulating factor serum levels correlate with the risk of infection in neutropenia due to Felty's syndrome or systemic lupus erythematosus. ( 12133752 )
2002
22
Cystic rheumatoid arthritis with Felty's syndrome and ankylosing spondylitis. ( 10380264 )
1999
23
Felty syndrome complicating juvenile rheumatoid arthritis. ( 9787332 )
1998
24
An unusual association of Felty syndrome and TCR gamma delta lymphocytosis. ( 8655718 )
1996
25
[Felty syndrome: a therapy-resistant variant of chronic rheumatoid arthritis? 2 case reports and literature review]. ( 8657998 )
1996
26
Treatment of the neutropenia of Felty syndrome. ( 8932830 )
1996
27
Large granular lymphocyte expansions in Felty's syndrome have an unusual phenotype of activated CD45RA+ cells. ( 9010052 )
1996
28
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
29
Incidence of cancer among men with the Felty syndrome. ( 8250454 )
1994
30
The Felty syndrome and G-CSF-associated thrombocytopenia and severe anemia. ( 7678374 )
1993
31
Oral methotrexate in the treatment of Felty's syndrome. ( 8478888 )
1993
32
GM-CSF in the treatment of Felty syndrome. ( 2024642 )
1991
33
The Felty syndrome: a case-matched study of clinical manifestations and outcome, serologic features, and immunogenetic associations. ( 1969604 )
1990
34
Molecular characterisation of C4 null alleles found in Felty's syndrome. ( 1978638 )
1990
35
Antihistone antibodies in rheumatoid arthritis and Felty's syndrome. ( 2803331 )
1989
36
Parenteral gold therapy in the Felty syndrome. Experience with 20 patients. ( 3951357 )
1986
37
Felty syndrome: autoimmune neutropenia or immune-complex-mediated disease? ( 3880180 )
1985
38
Felty syndrome in juvenile arthritis. ( 3968615 )
1985
39
Phagocytosis and intracellular killing by polymorphonuclear cells from patients with rheumatoid arthritis and Felty's syndrome. ( 3986006 )
1985
40
Felty's syndrome in a child. ( 6520839 )
1984
41
Felty's syndrome associated with high levels of IgA rheumatoid factor. ( 6742912 )
1984
42
Nodular regenerative hyperplasia of the liver and bleeding esophageal varices in Felty's syndrome: a case report and literature review. ( 6983577 )
1982
43
Felty's syndrome: response to parenteral gold. ( 7328565 )
1981
44
Felty syndrome. ( 7414334 )
1980
45
Felty's syndrome in blacks. ( 7362694 )
1980
46
Felty's syndrome. ( 159890 )
1979
47
Exodontics in a patient with Felty syndrome: report of case. ( 271700 )
1978
48
Improvement of rheumatoid arthritis following splenectomy for Felty syndrome. ( 300117 )
1977
49
The neutropenia of Felty's syndrome. ( 329671 )
1977
50
Decreased spleen size and increased neutrophils in patients with Felty syndrome. Effects of gold sodium thiomalate therapy. ( 818413 )
1976

Variations for Felty Syndrome

Expression for Felty Syndrome

Search GEO for disease gene expression data for Felty Syndrome.

Pathways for Felty Syndrome

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

(show all 28)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.65 C4A CSF2 CSF3 CXCL8 IL3 LTF
2
Show member pathways
13.33 CSF2 CSF3 CXCL8 IL3 TNFRSF10A
3
Show member pathways
13.06 CSF2 CSF3 CXCL8 IL3 TNFRSF10A
4
Show member pathways
13.03 CSF2 CSF3 CXCL8 IL3
5
Show member pathways
12.94 CSF2 CSF3 CXCL8 IL3 TNFRSF10A
6
Show member pathways
12.22 CSF2 IL3 TNFRSF10A
7
Show member pathways
12.1 CSF2 CSF3 IL3
8
Show member pathways
12.04 CSF2 CSF3 CXCL8
9 11.97 CSF2 CXCL8 IL3
10
Show member pathways
11.79 CXCL8 IL3 TNFRSF10A
11 11.7 CSF2 CSF3 IL3
12 11.62 CSF2 CXCL8
13 11.6 CSF2 CSF3 IL3
14 11.59 CSF2 CXCL8
15 11.56 CSF2 CXCL8
16
Show member pathways
11.55 CSF2 CSF3 CXCL8
17 11.54 CSF2 CXCL8
18 11.49 C4A CXCL8
19
Show member pathways
11.43 CSF2 CXCL8 IL3
20
Show member pathways
11.4 CSF2 CXCL8
21 11.3 CSF2 CSF3 CXCL8
22 11.27 CSF3 CXCL8
23 11.12 CSF2 CSF3 CXCL8
24 11.07 CSF2 CXCL8
25 11.01 CSF2 CSF3 IL3
26 10.7 CSF2 CSF3 CXCL8 IL3
27 10.49 CSF2 CSF3 CXCL8 IL3
28 10.26 CSF2 CSF3 IL3

GO Terms for Felty Syndrome

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 IL3 LTF
2 extracellular space GO:0005615 9.1 C4A CSF2 CSF3 CXCL8 IL3 LTF

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

# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.46 CSF2 CSF3 CXCL8 IL3
2 cellular response to lipopolysaccharide GO:0071222 9.43 CSF2 CSF3 CXCL8
3 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.4 CSF3 IL3
4 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.37 CSF2 IL3
5 antimicrobial humoral immune response mediated by antimicrobial peptide GO:0061844 9.32 CXCL8 LTF
6 regulation of signaling receptor activity GO:0010469 9.26 CSF2 CSF3 CXCL8 IL3
7 cytokine-mediated signaling pathway GO:0019221 8.92 CSF2 CSF3 CXCL8 IL3

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 for Felty Syndrome

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
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