MCID: FLT011
MIFTS: 51

Felty Syndrome

Categories: Blood diseases, Bone diseases, Immune diseases, Rare diseases

Aliases & Classifications for Felty Syndrome

MalaCards integrated aliases for Felty Syndrome:

Name: Felty Syndrome 57 12 20 58 44 70 32
Felty's Syndrome 12 73 20 15 17
Rheumatoid Arthritis with Splenoadenomegaly and Leukopenia 12
Splenomegaly-Neutropenia-Rheumatoid Arthritis Syndrome 58
Rheumatoid Arthritis, Splenomegaly and Neutropenia 20
Familial Felty's Syndrome 20
Feltys Syndrome 54

Characteristics:

Orphanet epidemiological data:

58
felty syndrome
Age of onset: Adult; Age of death: elderly;

OMIM®:

57 (Updated 05-Apr-2021)
Inheritance:
autosomal dominant


HPO:

31
felty syndrome:
Inheritance autosomal dominant inheritance


Classifications:

Orphanet: 58  
Rare systemic and rhumatological diseases
Rare immunological diseases


Summaries for Felty Syndrome

GARD : 20 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 ), Sjogren 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 large granular lymphocyte leukemia and aspergillosis. An important gene associated with Felty Syndrome is CSF3 (Colony Stimulating Factor 3), and among its related pathways/superpathways are Innate Immune System and JAK-STAT signaling pathway. The drugs Lenograstim and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include spleen, liver and bone, and related phenotypes are arthritis and immunodeficiency

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

Wikipedia : 73 Felty's syndrome, also called Felty syndrome, (FS) is rare autoimmune disease characterized by the triad... more...

More information 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 237)
# Related Disease Score Top Affiliating Genes
1 large granular lymphocyte leukemia 30.8 STAT3 CSF3
2 aspergillosis 30.4 STAT3 CSF3 CSF2
3 granulocytopenia 30.3 IL3 CSF3 CSF2
4 bacterial infectious disease 30.1 IL3 H2AC18 CSF3 CSF2 C4A
5 lymphoma, hodgkin, classic 30.0 IL3 CSF3 CSF2
6 t-cell large granular lymphocyte leukemia 30.0 STAT3 CSF2 B3GAT1
7 hypereosinophilic syndrome 29.9 STAT3 IL3 CSF2
8 allergic disease 29.9 STAT3 IL3 CSF2
9 cellulitis 29.8 CSF3 CSF2 CD8A
10 mature t-cell and nk-cell lymphoma 29.7 STAT3 H2AC18 CD8A
11 myeloid leukemia 29.7 STAT3 IL3 CSF3 CSF2
12 pancytopenia 29.6 IL3 H2AC18 CSF3 CSF2 CD8A
13 glomerulonephritis 29.3 PRTN3 C4B C4A
14 autoimmune disease 29.3 STAT3 PRTN3 PADI4 CSF2 C4A
15 exanthem 29.2 PRTN3 H2AC18 CSF3 CSF2 CD8A
16 systemic lupus erythematosus 29.1 STAT3 PRTN3 PADI4 H2AC18 CSF3 CSF2
17 lymphoma, non-hodgkin, familial 28.8 TNFRSF10A STAT3 IL3 H2AC18 CSF3 CSF2
18 neutropenia 10.5
19 splenomegaly 10.4
20 varicose veins 10.4
21 portal hypertension 10.4
22 nodular regenerative hyperplasia 10.4
23 esophageal varix 10.3
24 engraftment syndrome 10.3 CSF3 CSF2
25 vasculitis 10.2
26 retinitis pigmentosa and erythrocytic microcytosis 10.2 IL3 CSF3
27 intrinsic asthma 10.2 IL3 CSF2
28 mucormycosis 10.2 CSF3 CSF2
29 hypersplenism 10.2
30 juvenile dermatitis herpetiformis 10.2 C4B C4A
31 immunodeficiency due to a classical component pathway complement deficiency 10.2 C4B C4A
32 louse-borne relapsing fever 10.2 C4B C4A
33 relapsing fever 10.2 C4B C4A
34 tick-borne relapsing fever 10.2 C4B C4A
35 arthritis 10.2
36 neutrophilia, hereditary 10.2 IL3 CSF3 CSF2
37 metanephric adenoma 10.2 CSF3 B3GAT1
38 complement factor i deficiency 10.2 C4B C4A
39 eosinophilic gastroenteritis 10.1 IL3 CSF3 CSF2
40 gastroenteritis 10.1 IL3 CSF3 CSF2
41 neutropenia, severe congenital, 3, autosomal recessive 10.1 IL3 CSF3 CSF2
42 pediatric systemic lupus erythematosus 10.1 C4B C4A
43 cyclic neutropenia 10.1 IL3 CSF3 CSF2
44 aggressive nk-cell leukemia 10.1 STAT3 B3GAT1
45 epilepsy, familial temporal lobe, 2 10.1 C4B C4A
46 posterior scleritis 10.1 TNFRSF10A PRTN3
47 orbital granuloma 10.1 PRTN3 CSF3
48 mucositis 10.1 IL3 CSF3 CSF2
49 thrombocytosis 10.1 IL3 CSF3 CSF2
50 complement component 3 deficiency 10.1 C4B C4A

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

Human phenotypes related to Felty Syndrome:

58 31 (show all 38)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 arthritis 58 31 hallmark (90%) Very frequent (99-80%) HP:0001369
2 immunodeficiency 58 31 hallmark (90%) Very frequent (99-80%) HP:0002721
3 autoimmunity 58 31 hallmark (90%) Very frequent (99-80%) HP:0002960
4 subcutaneous nodule 58 31 hallmark (90%) Very frequent (99-80%) HP:0001482
5 arthralgia 58 31 hallmark (90%) Very frequent (99-80%) HP:0002829
6 neutropenia 58 31 hallmark (90%) Very frequent (99-80%) HP:0001875
7 limitation of joint mobility 58 31 hallmark (90%) Very frequent (99-80%) HP:0001376
8 osteolysis 58 31 hallmark (90%) Very frequent (99-80%) HP:0002797
9 synovitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0100769
10 splenomegaly 58 31 frequent (33%) Frequent (79-30%) HP:0001744
11 chronic otitis media 58 31 frequent (33%) Frequent (79-30%) HP:0000389
12 anemia 58 31 frequent (33%) Frequent (79-30%) HP:0001903
13 sinusitis 58 31 frequent (33%) Frequent (79-30%) HP:0000246
14 recurrent pharyngitis 58 31 frequent (33%) Frequent (79-30%) HP:0100776
15 weight loss 58 31 frequent (33%) Frequent (79-30%) HP:0001824
16 recurrent pneumonia 58 31 frequent (33%) Frequent (79-30%) HP:0006532
17 lymphadenopathy 58 31 frequent (33%) Frequent (79-30%) HP:0002716
18 rhinitis 58 31 frequent (33%) Frequent (79-30%) HP:0012384
19 abnormal lymphocyte morphology 31 frequent (33%) HP:0004332
20 hepatomegaly 58 31 occasional (7.5%) Occasional (29-5%) HP:0002240
21 thrombocytopenia 58 31 occasional (7.5%) Occasional (29-5%) HP:0001873
22 pulmonary fibrosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0002206
23 irregular hyperpigmentation 58 31 occasional (7.5%) Occasional (29-5%) HP:0007400
24 lymphoma 58 31 occasional (7.5%) Occasional (29-5%) HP:0002665
25 cellulitis 58 31 occasional (7.5%) Occasional (29-5%) HP:0100658
26 recurrent urinary tract infections 58 31 occasional (7.5%) Occasional (29-5%) HP:0000010
27 peripheral neuropathy 58 31 occasional (7.5%) Occasional (29-5%) HP:0009830
28 pericarditis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001701
29 sepsis 58 31 occasional (7.5%) Occasional (29-5%) HP:0100806
30 generalized hyperpigmentation 58 31 occasional (7.5%) Occasional (29-5%) HP:0007440
31 bone marrow hypocellularity 58 31 occasional (7.5%) Occasional (29-5%) HP:0005528
32 pleuritis 58 31 occasional (7.5%) Occasional (29-5%) HP:0002102
33 episcleritis 58 31 occasional (7.5%) Occasional (29-5%) HP:0100534
34 recurrent respiratory infections 58 Very frequent (99-80%)
35 recurrent infections 58 Very frequent (99-80%)
36 abnormal joint morphology 58 Very frequent (99-80%)
37 abnormality of lymphocytes 58 Frequent (79-30%)
38 rheumatoid arthritis 31 HP:0001370

Symptoms via clinical synopsis from OMIM®:

57 (Updated 05-Apr-2021)
G I:
splenomegaly

Joints:
rheumatoid arthritis

Heme:
neutropenia

Misc:
confined mainly to white males
hla-drw4 association

Clinical features from OMIM®:

134750 (Updated 05-Apr-2021)

MGI Mouse Phenotypes related to Felty Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.65 C4B CD8A CSF2 CSF3 EEF1A1 PADI2
2 immune system MP:0005387 9.32 C4B CD8A CSF2 CSF3 EEF1A1 PADI2

Drugs & Therapeutics for Felty Syndrome

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
Lenograstim Approved, Investigational Phase 2 135968-09-1
2
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
3 Alkylating Agents Phase 2
4 Immunosuppressive Agents Phase 2
5 Immunologic Factors Phase 2
6 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

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:

40
Spleen, Liver, Bone, Bone Marrow, Neutrophil, Skin, Myeloid

Publications for Felty Syndrome

Articles related to Felty Syndrome:

(show top 50) (show all 733)
# Title Authors PMID Year
1
Cloning and expression of a novel human antibody-antigen pair associated with Felty's syndrome. 57 61
10922075 2000
2
HLA-DRw4 in Felty's syndrome. 61 57
6934775 1980
3
Felty's syndrome in blacks. 57 61
7362694 1980
4
The rarity of Felty's syndrome in blacks. 57 61
475875 1979
5
Familial Felty's syndrome. 57 61
984910 1976
6
Autoantibodies against granulocyte-macrophage colony stimulating factor and interleukin-3 are rare in patients with Felty's syndrome. 54 61
15194585 2004
7
Autoantibodies against granulocyte colony-stimulating factor in Felty's syndrome and neutropenic systemic lupus erythematosus. 61 54
12355486 2002
8
Chronic neutropenia associated with autoimmune disease. 54 61
11957195 2002
9
[G-CSF versus GM-CSF in the treatment of neutropenia in a patient with Felty's syndrome on hemodialysis]. 61 54
11100668 2000
10
Primary aspergillosis of the larynx in a patient with Felty's syndrome. 54 61
10949734 2000
11
Treatment of severe neutropenia due to Felty's syndrome or systemic lupus erythematosus with granulocyte colony-stimulating factor. 54 61
10553980 1999
12
Treatment of Felty's syndrome with the haemopoietic growth factor granulocyte colony-stimulating factor (G-CSF). 54 61
9519212 1998
13
The use of granulocyte colony-stimulating factor in joint replacement surgery in a patient with Felty's syndrome. 54 61
9402873 1997
14
Prolonged treatment with granulocyte colony-stimulating factor in a patient with Felty's syndrome and chronic renal failure from secondary amyloidosis. 61 54
9269662 1997
15
Felty's Syndrome. 61 54
18031099 1997
16
Use of colony-stimulating factors in the treatment of neutropenia associated with collagen vascular disease. 54 61
9209836 1997
17
Successful Treatment of Perianal Ulcers in a Patient with Felty's Syndrome Using Granulocyte Colony Stimulating Factor. 54 61
19078084 1996
18
An unusual association of Felty syndrome and TCR gamma delta lymphocytosis. 61 54
8655718 1996
19
Effects of antirheumatic agents on cytokines. 54 61
8834013 1996
20
Vasculitis complicating granulocyte colony stimulating factor treatment of leukopenia and infection in Felty's syndrome. 54 61
7545756 1995
21
Reactivation of rheumatoid arthritis and development of leukocytoclastic vasculitis in a patient receiving granulocyte colony-stimulating factor for Felty's syndrome. 61 54
7539978 1995
22
Flare of arthritis with successful treatment of Felty's syndrome with granulocyte colony stimulating factor (GCSF). 61 54
7540528 1995
23
Combined therapy of G-CSF and prednisolone for neutropenia in a patient with Felty's syndrome. 61 54
7531393 1995
24
A prolonged use of granulocyte colony stimulating factor in Felty's syndrome. 61 54
7699667 1995
25
G-CSF in Felty's syndrome: correction of neutropenia and effects on cytokine release. 54 61
7538060 1995
26
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. 54 61
7524509 1994
27
Successful reversal of neutropenia in Felty's syndrome with recombinant granulocyte colony stimulating factor. 61 54
7519040 1994
28
CD4+ T cell-mediated leukopenia of Felty's syndrome successfully treated with granulocyte-colony-stimulating factor and methotrexate. 61 54
7510488 1994
29
Granulocyte colony-stimulating factor induction of improved leukocytopenia with inflammatory flare in a Felty's syndrome patient. 54 61
7510482 1994
30
Sequential administration of cyclophosphamide and granulocyte-colony stimulating factor relieves impaired myeloid maturation in Felty's syndrome. 54 61
7690520 1993
31
Long-term correction of neutropenia in Felty's syndrome with granulocyte colony-stimulating factor. 61 54
7685194 1993
32
G-CSF improves granulocytopenia in Felty's syndrome without flare-up of arthritis. 54 61
7679881 1993
33
GM-CSF and G-CSF in Felty's syndrome. 54 61
7681711 1993
34
Treatment of neutropenia in Felty's syndrome with granulocyte-macrophage colony-stimulating factor--hematological response accompanied by pulmonary complications with lethal outcome. 61 54
1457582 1992
35
Granulocyte-colony stimulating factor corrects granulocytopenia in Felty's syndrome. 61 54
1380205 1992
36
GM-CSF versus G-CSF in the treatment of infectious complication in Felty's syndrome--a case report. 54 61
1374651 1992
37
Felty's syndrome: favorable response to granulocyte-macrophage colony-stimulating factor in the acute phase. 61 54
1519433 1992
38
GM-CSF in the treatment of Felty syndrome. 61 54
2024642 1991
39
Improvement of pneumonia and arthritis in Felty's syndrome by treatment with granulocyte-macrophage colony-stimulating factor (GM-CSF). 61 54
2291988 1990
40
Granulocyte-macrophage colony-stimulating factor treatment of neutropenia associated with Felty's syndrome. 61 54
2261008 1990
41
Molecular characterisation of C4 null alleles found in Felty's syndrome. 61 54
1978638 1990
42
Delayed Tracheal Perforation Following Total Thyroidectomy. 61
33782958 2021
43
The role of intravenous immunoglobulin in treatment of refractory Felty syndrome c. 61
33631851 2021
44
Analysis of a single-institution cohort of patients with Felty's syndrome and T-cell large granular lymphocytic leukemia in the setting of rheumatoid arthritis. 61
33280072 2021
45
Rheumatoid arthritis patients with peripheral blood cell reduction should be evaluated for latent Felty syndrome: A case report. 61
33371095 2020
46
Concomitant Large Loculated Pleural and Pericardial Effusions in a Patient with Rheumatoid Arthritis on Methotrexate. 61
33482962 2020
47
Hematological Manifestations among Patients with Rheumatic Diseases. 61
33221805 2020
48
Successful abatacept treatment for Felty's syndrome in a patient with rheumatoid arthritis. 61
33086993 2020
49
Tocilizumab treatment in Felty's syndrome. 61
32347340 2020
50
The Diagnostic Conundrum of Felty Syndrome and T-Cell Large Granular Lymphocytic Leukemia. 61
32530865 2020

Variations for Felty Syndrome

Expression for Felty Syndrome

Search GEO for disease gene expression data for Felty Syndrome.

Pathways for Felty Syndrome

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 space GO:0005615 9.5 PRTN3 IL3 EEF1A1 CSF3 CSF2 C4B
2 extracellular region GO:0005576 9.32 PRTN3 PADI2 IL3 EEF1A1 CSF3 CSF2

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

# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.8 STAT3 PRTN3 IL3 CSF3 CSF2
2 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.54 STAT3 IL3 CSF2
3 positive regulation of apoptotic cell clearance GO:2000427 9.32 C4B C4A
4 histone citrullination GO:0036414 9.26 PADI4 PADI2
5 protein citrullination GO:0018101 9.16 PADI4 PADI2
6 cytokine-mediated signaling pathway GO:0019221 9.02 STAT3 PRTN3 IL3 CSF3 CSF2
7 histone H3-R26 citrullination GO:0036413 8.96 PADI4 PADI2

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 IL3 CSF3 CSF2
2 protein-arginine deiminase activity GO:0004668 8.62 PADI4 PADI2

Sources for Felty Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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