MCID: IND002
MIFTS: 42

Indolent Systemic Mastocytosis

Categories: Blood diseases, Cancer diseases, Rare diseases

Aliases & Classifications for Indolent Systemic Mastocytosis

MalaCards integrated aliases for Indolent Systemic Mastocytosis:

Name: Indolent Systemic Mastocytosis 12 60 15 74
Ism 12

Characteristics:

Orphanet epidemiological data:

60
indolent systemic mastocytosis
Inheritance: Not applicable; Prevalence: 1-9/100000 (Europe); Age of onset: All ages;

Classifications:



External Ids:

Disease Ontology 12 DOID:4660
MeSH 45 D034721
NCIt 51 C9286
SNOMED-CT 69 70910003
ICD10 34 D47.02
ICD10 via Orphanet 35 D47.0
UMLS via Orphanet 75 C0272203
Orphanet 60 ORPHA98848
UMLS 74 C0272203

Summaries for Indolent Systemic Mastocytosis

MalaCards based summary : Indolent Systemic Mastocytosis, also known as ism, is related to systemic mastocytosis and mastocytosis, cutaneous. An important gene associated with Indolent Systemic Mastocytosis is KIT (KIT Proto-Oncogene, Receptor Tyrosine Kinase), and among its related pathways/superpathways are Akt Signaling and PAK Pathway. The drugs Cladribine and Histamine Antagonists have been mentioned in the context of this disorder. Affiliated tissues include bone, skin and bone marrow, and related phenotypes are sudden cardiac death and nausea and vomiting

Related Diseases for Indolent Systemic Mastocytosis

Graphical network of the top 20 diseases related to Indolent Systemic Mastocytosis:



Diseases related to Indolent Systemic Mastocytosis

Symptoms & Phenotypes for Indolent Systemic Mastocytosis

Human phenotypes related to Indolent Systemic Mastocytosis:

60 33 (show all 13)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 sudden cardiac death 60 33 hallmark (90%) Very frequent (99-80%) HP:0001645
2 nausea and vomiting 60 33 hallmark (90%) Very frequent (99-80%) HP:0002017
3 abdominal pain 60 33 hallmark (90%) Very frequent (99-80%) HP:0002027
4 pruritus 60 33 hallmark (90%) Very frequent (99-80%) HP:0000989
5 headache 60 33 hallmark (90%) Very frequent (99-80%) HP:0002315
6 diarrhea 60 33 hallmark (90%) Very frequent (99-80%) HP:0002014
7 urticaria 60 33 hallmark (90%) Very frequent (99-80%) HP:0001025
8 abnormality of skin pigmentation 60 33 hallmark (90%) Very frequent (99-80%) HP:0001000
9 mastocytosis 60 33 hallmark (90%) Very frequent (99-80%) HP:0100495
10 impaired temperature sensation 60 33 hallmark (90%) Very frequent (99-80%) HP:0010829
11 behavioral abnormality 60 33 frequent (33%) Frequent (79-30%) HP:0000708
12 reduced bone mineral density 60 33 occasional (7.5%) Occasional (29-5%) HP:0004349
13 abnormality of the gastric mucosa 60 33 occasional (7.5%) Occasional (29-5%) HP:0004295

Drugs & Therapeutics for Indolent Systemic Mastocytosis

Drugs for Indolent Systemic Mastocytosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 18)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cladribine Approved, Investigational Phase 3 4291-63-8 20279
2 Histamine Antagonists Phase 3,Phase 2
3 Interferon-alpha Phase 3
4 Histamine H1 Antagonists Phase 3
5 Cromolyn Sodium Phase 3,Phase 2
6 Leukotriene Antagonists Phase 3
7 2-chloro-3'-deoxyadenosine Phase 3
8 Antidepressive Agents Phase 3
9 Proton Pump Inhibitors Phase 3
10 interferons Phase 3
11
midostaurin Approved, Investigational Phase 2 120685-11-2 9829523 104937
12
Tyrosine Approved, Investigational, Nutraceutical Phase 2 60-18-4 6057
13 Staurosporine Experimental Phase 2 62996-74-1
14 Specific substance maruyama Phase 2
15 Protein Kinase Inhibitors Phase 2
16 Antibodies Phase 2
17 Antibodies, Monoclonal Phase 2
18 Immunoglobulins Phase 2

Interventional clinical trials:

(show all 11)
# Name Status NCT ID Phase Drugs
1 Masitinib in Severe Indolent or Smoldering Systemic Mastocytosis Completed NCT00814073 Phase 3 Masitinib;Placebo
2 Study on Mastocytosis for Rupatadine Treatment Completed NCT01481909 Phase 2, Phase 3 Rupatadin
3 Midostaurin in Indolent Systemic Mastocytosis Unknown status NCT01920204 Phase 2 Midostaurin,
4 Treatment of Indolent Systemic Mastocytosis With PA101 Completed NCT02478957 Phase 2 PA101;Placebo
5 Efficacy of AB1010 in Patients With Systemic Indolent Mastocytosis Completed NCT00831974 Phase 2 masitinib (AB1010);masitinib (AB1010)
6 Safety and Efficacy of Subcutaneous Sarilumab in Improving the Quality of Life in People With Indolent Systemic Mastocytosis Recruiting NCT03770273 Phase 2
7 (PIONEER) Study to Evaluate Efficacy and Safety of Avapritinib (BLU-285), A Selective KIT Mutation-targeted Tyrosine Kinase Inhibitor, Versus Placebo in Patients With Indolent and Smoldering Systemic Mastocytosis Recruiting NCT03731260 Phase 2 Avapritinib;Placebo
8 A Study to Evaluate the Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of AK002 Completed NCT02808793 Phase 1 AK002
9 Patient-Reported Outcome Questionnaire for Systemic Mastocytosis Completed NCT02380222
10 Mast Cell Connect: A Registry for Patients With Mastocytosis Recruiting NCT02620254
11 Adaptation of the Questionnaire "Regarding Patient's Quality of Life With Mastocytosis" in the French Language Not yet recruiting NCT03632811

Search NIH Clinical Center for Indolent Systemic Mastocytosis

Genetic Tests for Indolent Systemic Mastocytosis

Anatomical Context for Indolent Systemic Mastocytosis

MalaCards organs/tissues related to Indolent Systemic Mastocytosis:

42
Bone, Skin, Bone Marrow, Lung, Colon, Myeloid

Publications for Indolent Systemic Mastocytosis

Articles related to Indolent Systemic Mastocytosis:

(show top 50) (show all 125)
# Title Authors Year
1
Indolent systemic mastocytosis mimicking Crohn's disease. ( 30872912 )
2019
2
Omalizumab Therapy for Mast Cell-Mediator Symptoms in Patients with ISM, CM, MMAS, and MCAS. ( 30954641 )
2019
3
Bend and Moisture Effects on the Performance of a U-Shaped Slotted Wearable Antenna for Off-Body Communications in an Industrial Scientific Medical (ISM) 2.4 GHz Band. ( 30991753 )
2019
4
HCnN anions in the ISM: exploring their existence and new paths to anionic carbonitriles for n = 3, 5. ( 31111134 )
2019
5
Indolent Systemic Mastocytosis Manifesting as Protracted Anaphylactic Shock. ( 29743774 )
2018
6
Midostaurin in patients with indolent systemic mastocytosis: An open-label phase 2 trial. ( 29890238 )
2018
7
An interpretive structural modeling (ISM) and decision-making trail and evaluation laboratory (DEMATEL) method approach for the analysis of barriers of waste recycling in India. ( 28278038 )
2018
8
Determination of a new index of sexual maturity (ISM) in zebra mussel using flow cytometry: interest in ecotoxicology. ( 28567680 )
2018
9
A phase II study to evaluate the pharmacokinetics, safety, and tolerability of Risperidone ISM multiple intramuscular injections once every 4 weeks in patients with schizophrenia. ( 29112001 )
2018
10
A Call for a New Ism in Pharmacy. ( 29606715 )
2018
11
The cytoskeleton in 'couch potato-ism': Insights from a murine model of impaired actin dynamics. ( 29684438 )
2018
12
Factors affecting strategic plan implementation using interpretive structural modeling (ISM). ( 29865959 )
2018
13
Optimal Design of a Planar Textile Antenna for Industrial Scientific Medical (ISM) 2.4 GHz Wireless Body Area Networks (WBAN) with the CRO-SL Algorithm. ( 29933585 )
2018
14
Paper-Based Flexible Antenna for Wearable Telemedicine Applications at 2.4 GHz ISM Band. ( 30513719 )
2018
15
Longitudinal Evaluation of Bone Mineral Density and Bone Metabolism Markers in Patients with Indolent Systemic Mastocytosis Without Osteoporosis. ( 27743017 )
2017
16
Masitinib for treatment of severely symptomatic indolent systemic mastocytosis: a randomised, placebo-controlled, phase 3 study. ( 28069279 )
2017
17
Kounis Syndrome During Anesthesia: Presentation of Indolent Systemic Mastocytosis: A Case Report. ( 28181948 )
2017
18
Routine abdominal ultrasonography has limited value in the care for patients with indolent systemic mastocytosis. ( 28486845 )
2017
19
Management of poorly controlled indolent systemic mastocytosis using narrowband UVB phototherapy. ( 28632811 )
2017
20
The importance of health(ism): A focus group study of lesbian, gay, bisexual, pansexual, queer and transgender individuals' understandings of health. ( 26311816 )
2017
21
Explaining recurring maser flares in the ISM through large-scale entangled quantum mechanical states. ( 28378015 )
2017
22
Biodegradation of anthracene by a newly isolated bacterial strain, Bacillus thuringiensis AT.ISM.1, isolated from a fly ash deposition site. ( 28763108 )
2017
23
An Enhanced Robust Control Algorithm Based on CNF and ISM for the MEMS Micromirror against Input Saturation and Disturbance. ( 30400516 )
2017
24
Increased IL6 plasma levels in indolent systemic mastocytosis patients are associated with high risk of disease progression. ( 26153655 )
2016
25
Response of patients with indolent systemic mastocytosis to tamoxifen citrate. ( 26612479 )
2016
26
KIT D816V-mutated bone marrow mesenchymal stem cells in indolent systemic mastocytosis are associated with disease progression. ( 26622064 )
2016
27
Phase I, open-label, randomized, parallel study to evaluate the pharmacokinetics, safety, and tolerability of one intramuscular injection of risperidone ISM at different dose strengths in patients with schizophrenia or schizoaffective disorder (PRISMA-1). ( 27416102 )
2016
28
Ageism: The next big '-ism' to address? ( 27010872 )
2016
29
Face-ism and Objectification in Mainstream and LGBT Magazines. ( 27074012 )
2016
30
Implementation and Analysis of ISM 2.4 GHz Wireless Sensor Network Systems in Judo Training Venues. ( 27509501 )
2016
31
Evaluating the More Suitable ISM Frequency Band for IoT-Based Smart Grids: A Quantitative Study of 915 MHz vs. 2400 MHz. ( 28042863 )
2016
32
Meandered conformal antenna for ISM-band ingestible capsule communication systems. ( 28324976 )
2016
33
Response to celecoxib in a patient with indolent systemic mastocytosis presenting with intractable diarrhea. ( 26505934 )
2015
34
Sustained improvement in urticaria pigmentosa and pruritus in a case of indolent systemic mastocytosis treated with cladribine. ( 25515765 )
2015
35
MR-based trabecular bone microstructure is not altered in subjects with indolent systemic mastocytosis. ( 25542754 )
2015
36
The impact of sensitive KIT D816V detection on recognition of indolent Systemic Mastocytosis. ( 25582384 )
2015
37
Therapeutic challenge during the long-term follow-up of a patient with indolent systemic mastocytosis with extensive cutaneous involvement. ( 26004600 )
2015
38
A 0.33 nJ/bit IEEE802.15.6/Proprietary MICS/ISM Wireless Transceiver With Scalable Data Rate for Medical Implantable Applications. ( 25807573 )
2015
39
Face-ism and kernels of truth in facial inferences. ( 26021212 )
2015
40
Tau-ism: The Yin and Yang of Microtubule Sliding, Detachment, and Rupture. ( 26636932 )
2015
41
Indolent systemic mastocytosis in a patient with ileocolitis. ( 24975749 )
2014
42
Omalizumab induced remission of idiopathic anaphylaxis in a patient suffering from indolent systemic mastocytosis. ( 24162824 )
2014
43
Serum levels of bone cytokines in indolent systemic mastocytosis associated with osteopenia or osteoporosis. ( 24439083 )
2014
44
Predictors of new fragility fractures after diagnosis of indolent systemic mastocytosis. ( 24985401 )
2014
45
Delayed diagnosis of adult indolent systemic mastocytosis. ( 25386326 )
2014
46
Endoscopic findings of indolent systemic mastocytosis involving the colon. ( 25526423 )
2014
47
Design and analysis of an implantable CPW-fed X-monopole antenna for 2.45-GHz ISM band applications. ( 24404821 )
2014
48
Wireless electrocardiogram transmission in ISM band: an approach towards telecardiology. ( 25085695 )
2014
49
Cold condensation of dust in the ISM. ( 25302393 )
2014
50
Analysis of radio wave propagation for ISM 2.4 GHz Wireless Sensor Networks in inhomogeneous vegetation environments. ( 25513820 )
2014

Variations for Indolent Systemic Mastocytosis

Expression for Indolent Systemic Mastocytosis

Search GEO for disease gene expression data for Indolent Systemic Mastocytosis.

Pathways for Indolent Systemic Mastocytosis

GO Terms for Indolent Systemic Mastocytosis

Biological processes related to Indolent Systemic Mastocytosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 male gonad development GO:0008584 9.37 KIT KITLG
2 positive regulation of MAP kinase activity GO:0043406 9.32 KIT KITLG
3 ovarian follicle development GO:0001541 9.26 KIT KITLG
4 positive regulation of cell proliferation GO:0008284 9.26 CLEC11A KIT KITLG MVD
5 embryonic hemopoiesis GO:0035162 9.16 KIT KITLG
6 ectopic germ cell programmed cell death GO:0035234 8.62 KIT KITLG

Molecular functions related to Indolent Systemic Mastocytosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 carboxy-lyase activity GO:0016831 8.62 HDC MVD

Sources for Indolent Systemic Mastocytosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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