MCID: SML040
MIFTS: 13

Smoldering Systemic Mastocytosis

Categories: Blood diseases, Rare diseases

Aliases & Classifications for Smoldering Systemic Mastocytosis

MalaCards integrated aliases for Smoldering Systemic Mastocytosis:

Name: Smoldering Systemic Mastocytosis 58

Classifications:

Orphanet: 58  
Rare haematological diseases


External Ids:

ICD10 via Orphanet 33 C96.2
Orphanet 58 ORPHA158775

Summaries for Smoldering Systemic Mastocytosis

MalaCards based summary : Smoldering Systemic Mastocytosis is related to systemic mastocytosis and mastocytosis. An important gene associated with Smoldering Systemic Mastocytosis is KIT (KIT Proto-Oncogene, Receptor Tyrosine Kinase). The drugs Cladribine and 2-chloro-3'-deoxyadenosine have been mentioned in the context of this disorder. Affiliated tissues include bone and bone marrow.

Related Diseases for Smoldering Systemic Mastocytosis

Diseases related to Smoldering Systemic Mastocytosis via text searches within MalaCards or GeneCards Suite gene sharing:

(showing 3, show less)
# Related Disease Score Top Affiliating Genes
1 systemic mastocytosis 10.1
2 mastocytosis 10.1
3 mast-cell leukemia 10.1

Symptoms & Phenotypes for Smoldering Systemic Mastocytosis

Drugs & Therapeutics for Smoldering Systemic Mastocytosis

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

(showing 20, show less)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cladribine Approved, Investigational Phase 3 4291-63-8 20279
2 2-chloro-3'-deoxyadenosine Phase 3
3 Proton Pump Inhibitors Phase 3
4 Histamine Antagonists Phase 3
5 Leukotriene Antagonists Phase 3
6 Histamine H1 Antagonists Phase 3
7 Cromolyn Sodium Phase 3
8 Antidepressive Agents Phase 3
9 interferons Phase 3
10 Interferon-alpha Phase 3
11
midostaurin Approved, Investigational Phase 2 120685-11-2 104937 9829523
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 Anti-Bacterial Agents Phase 2
17 Angiogenesis Inhibitors Phase 2
18 Anti-Infective Agents Phase 2
19 Immunosuppressive Agents Phase 2
20 Immunologic Factors Phase 2

Interventional clinical trials:

(showing 5, show less)
# Name Status NCT ID Phase Drugs
1 Randomized, Placebo-controlled, Phase 3 Study to Compare Efficacy and Safety of Masitinib at 6 mg/kg/Day to Placebo in Treatment of Patients With Smouldering Systemic, Indolent Systemic or Cutaneous Mastocytosis With Handicap Completed NCT00814073 Phase 3 Masitinib;Placebo
2 Phase II Single Arm Open Pilot Study to Demonstrate the Efficacy of Midostaurin in Symptom Improvement and Decrease of Mast Cell Burden in Patients With Indolent or Smoldering Systemic Mastocytosis. Unknown status NCT01920204 Phase 2 Midostaurin,
3 Phase II Study of Thalidomide in Mastocytosis Completed NCT00769587 Phase 2 thalidomide
4 A 3-Part, Randomized, Double-Blind, Placebo-Controlled Phase 2 Study to Evaluate Safety and Efficacy of Avapritinib (BLU-285), a Selective KIT Mutation-Targeted Tyrosine Kinase Inhibitor, in Indolent and Smoldering Systemic Mastocytosis With Symptoms Inadequately Controlled With Standard Therapy Recruiting NCT03731260 Phase 2 Avapritinib;Placebo
5 Mast Cell Connect: A Registry for Patients With Mastocytosis Recruiting NCT02620254

Search NIH Clinical Center for Smoldering Systemic Mastocytosis

Genetic Tests for Smoldering Systemic Mastocytosis

Anatomical Context for Smoldering Systemic Mastocytosis

MalaCards organs/tissues related to Smoldering Systemic Mastocytosis:

40
Bone, Bone Marrow

Publications for Smoldering Systemic Mastocytosis

Articles related to Smoldering Systemic Mastocytosis:

(showing 5, show less)
# Title Authors PMID Year
1
Systemic mastocytosis associated with myelodysplastic/myeloproliferative neoplasms with ring sideroblasts and thrombocytosis: Report of three cases. 61
31523839 2019
2
Novel approaches to treating advanced systemic mastocytosis. 61
31372066 2019
3
Diagnostic value of tryptase in anaphylaxis and mastocytosis. 61
16931288 2006
4
Bone marrow mast cell immunophenotyping in adults with mast cell disease: a prospective study of 33 patients. 61
15203275 2004
5
["Smoldering systemic mastocytosis. "Successful therapy with cladribine]. 61
15241520 2004

Variations for Smoldering Systemic Mastocytosis

Expression for Smoldering Systemic Mastocytosis

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

Pathways for Smoldering Systemic Mastocytosis

GO Terms for Smoldering Systemic Mastocytosis

Sources for Smoldering Systemic Mastocytosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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