Smoldering Systemic Mastocytosis disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials

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Aliases & Classifications for Smoldering Systemic Mastocytosis

MalaCards integrated aliases for Smoldering Systemic Mastocytosis:

Name: Smoldering Systemic Mastocytosis ⁵⁸

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Blood diseases

See all MalaCards categories (disease lists)

ICD10: ³³

Neoplasms

Malignant neoplasms, stated or presumed to be primary, of lymphoid, haematopoietic and related tissue

Other and unspecified malignant neoplasms of lymphoid, haematopoietic and related tissue

Malignant mast cell tumour

Orphanet: ⁵⁸

Rare haematological diseases

External Ids:

ICD10 via Orphanet ³³ C96.2

Orphanet ⁵⁸ ORPHA158775

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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 marrow.

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Related Diseases for Smoldering Systemic Mastocytosis

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

#	Related Disease	Score
1	systemic mastocytosis	10.1
2	mastocytosis	10.1
3	mast-cell leukemia	10.1

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Symptoms & Phenotypes for Smoldering Systemic Mastocytosis

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Drugs & Therapeutics for Smoldering Systemic Mastocytosis

Drugs for Smoldering 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

Synonyms:

(2R,3S,5R)-5-(6-amino-2-chloropurin-9-yl)-2-(hydroxymethyl)oxolan-3-ol

(2R,3S,5R)-5-(6-Amino-2-chloropurin-9-yl)-2-(hydroxymethyl)oxolan-3-ol

2 Chlorodeoxyadenosine

24757-90-2

2CdA

2-CdA

2-Chloro-2'-deoxyadenosine

2-Chloro-2'-deoxy-b-adenosine

2-Chloro-2'-deoxy-beta-adenosine

2-Chloro-2'-deoxy-β-adenosine

2-Chloro-6-amino-9-(2-deoxy-b-D-erythro-pentofuranosyl)purine

2-chloro-6-amino-9-(2-deoxy-beta-D-erythro-pentofuranosyl)purine

2-Chloro-6-amino-9-(2-deoxy-beta-D-erythropentofuranosyl)purine

2-Chloro-6-amino-9-(2-deoxy-beta-D-erythro-pentofuranosyl)purine

2-chloro-6-amino-9-(2-deoxy-β-D-erythro-pentofuranosyl)purine

2-Chloro-6-amino-9-(2-deoxy-β-D-erythro-pentofuranosyl)purine

2-chloro-deoxyadenosine

2-Chlorodeoxyadenosine

2-Chloro-deoxyadenosine

2ClAdo

2'-Deoxy-2-chloroadenosine

4291-63-8

AC1L2FXP

AC-7591

Adenosine, 2-chloro-2'-deoxy

BRN 0624220

C10H12ClN5O3.C3H8

CHEBI:567361

CHEMBL1619

Chlorodeoxyadenosine

CID20279

CL9

Cladarabine

Cladaribine

cladribina

Cladribina

cladribine

Cladribine

Cladribine (JAN/USAN/INN)

Cladribine [USAN:INN:BAN]

cladribinum

Cladribinum

CldAdo

CPD000058553

D01370

D017338

DB00242

FT-0080707

HMS2052K13

HSDB 7564

Leustat

Leustatin

Leustatin (TN)

Leustatin, 2-chlorodeoxyadenosine, Cladribine

Litak

LS-15109

Mavenclad

MLS000028377

MLS000028484

MLS000759397

MLS001077345

MolPort-002-054-532

MolPort-005-935-074

Movectro

Mylinax

NCGC00022567-05

NCGC00164384-01

NSC 105014

NSC 105014-F

NSC-105014

RWJ 26251

RWJ-26251

RWJ-26251-000

S1199_Selleck

SAM001246526

SMR000058553

UNII-47M74X9YT5

ZINC03798064

2

2-chloro-3'-deoxyadenosine

Phase 3

3

Interferon-alpha

Phase 3

4

interferons

Phase 3

5

Proton Pump Inhibitors

Phase 3

6

Histamine Antagonists

Phase 3

7

Leukotriene Antagonists

Phase 3

8

Cromolyn Sodium

Phase 3

9

Antidepressive Agents

Phase 3

10

Histamine H1 Antagonists

Phase 3

11

midostaurin

Approved, Investigational

Phase 2

120685-11-2

104937 9829523

Synonyms:

120685-11-2

4'-N-benzoylstaurosporine

C411007

CHEMBL338448

CHEMBL608533

CID9829523

CPG 41251

D05029

HMS3229K17

midostaurin

Midostaurin

Midostaurin (USAN/INN)

N-Benzoylstaurosporine

NSC656576

PKC412

PKC-412

RYDAPT

12

Tyrosine

Approved, Investigational, Nutraceutical

Phase 2

60-18-4

6057

Synonyms:

(-)-a-Amino-p-hydroxyhydrocinnamate

(-)-a-Amino-p-hydroxyhydrocinnamic acid

(-)-alpha-Amino-p-hydroxyhydrocinnamate

(-)-alpha-Amino-p-hydroxyhydrocinnamic acid

(-)-Α-amino-p-hydroxyhydrocinnamate

(−)-α-amino-p-hydroxyhydrocinnamic acid

(-)-Α-amino-p-hydroxyhydrocinnamic acid

(2S)-2-Amino-3-(4-hydroxyphenyl)propanoate

(2S)-2-amino-3-(4-hydroxyphenyl)propanoic acid

(2S)-2-Amino-3-(4-hydroxyphenyl)propanoic acid

(S)-(-)-Tyrosine

(S)-2-Amino-3-(4-hydroxyphenyl)propionic acid

(S)-2-Amino-3-(p-hydroxyphenyl)propionate

(S)-2-Amino-3-(p-hydroxyphenyl)propionic acid

(S)-3-(p-Hydroxyphenyl)alanine

(S)-a-Amino-4-hydroxybenzenepropanoate

(S)-a-Amino-4-hydroxy-benzenepropanoate

(S)-a-Amino-4-hydroxybenzenepropanoic acid

(S)-a-Amino-4-hydroxy-benzenepropanoic acid

(S)-alpha-Amino-4-hydroxybenzenepropanoate

(S)-alpha-Amino-4-hydroxy-benzenepropanoate

(S)-alpha-amino-4-Hydroxybenzenepropanoic acid

(S)-alpha-Amino-4-hydroxybenzenepropanoic acid

(S)-alpha-Amino-4-hydroxy-benzenepropanoic acid

(S)-Tyrosine

(S)-Α-amino-4-hydroxybenzenepropanoate

(S)-α-amino-4-hydroxybenzenepropanoic acid

(S)-Α-amino-4-hydroxybenzenepropanoic acid

140-43-2

1991-85-1

25619-78-7

2-Amino-3-(4-hydroxyphen yl)-2-amino-3-(4-hydroxyphenyl)-propanoate

2-Amino-3-(4-hydroxyphen yl)-2-amino-3-(4-hydroxyphenyl)-propanoic acid

2-Amino-3-(p-hydroxyphenyl)propionic acid

2csm

3-(4-Hydroxyphenyl)-L-alanine

3-(p-Hydroxyphenyl)alanine

46209-14-7

4-hydroxy-L-phenylalanine

4-Hydroxy-L-phenylalanine

4ts1

55520-40-6

60-18-4

93829_FLUKA

93829_SIGMA

AB1002647

AC-11295

AC1L1LPI

AC1Q4U7L

AC1Q4U7M

AI3-09055

alpha-Amino-beta-(4-hydroxyphenyl)propionic acid

AR-1J3457

BB_NC-1194

Benzenepropanoate

Benzenepropanoic acid

beta-(p-Hydroxyphenyl)alanine

bmse000051

C00082

CHEBI:17895

CHEMBL925

CID6057

D00022

DB00135

DB03839

DD69927C-C6A8-4BC6-8E9A-0AB423B176E7

DTY

EINECS 200-460-4

FEMA No. 3736

Free-Form L-Tyrosine

HSDB 2003

H-Tyr-OH

L Tyrosine

L-(-)-Tyrosine

L-2-Amino-3-p-hydroxyphenylpropanoic acid

L-p-Tyrosine

LS-2336

L-Tyrosin

L-tyrosine

L-Tyrosine (9CI)

L-Tyrosine (JAN)

L-Tyrosine hydrochloride

L-Tyrosine, homopolymer

L-Tyrosine, monomer

Melanin synthesized from Tyr substrate catalyzed by tyrosinase for 6 hrs

Melanin synthesized from Tyr substrate catalyzed by tyrosinase for 6 hrs, oxidized with hydrogen peroxide

Melanin synthesized from Tyr substrate catalyzed by tyrosinase for 6 hrs, oxidized with hydrogen peroxide, <3 kd fraction

Melanin synthesized from Tyr substrate catalyzed by tyrosinase, brominated with N-bromosuccinimide

Melanin synthesized from Tyr substrate catalyzed by tyrosinase, sulfonated using sulfur trioxide/DMF complex for 1.5-7 hours

MolPort-001-792-016

NCGC00159350-02

NCGC00159350-03

nchembio.105-comp1

nchembio.121-comp7

nchembio.186-comp84

nchembio.284-comp7

nchembio816-comp11

NSC 82624

Para tyrosine

Para-tyrosine

p-Tyrosine

Rxosine

T0550

T3754_SIAL

T4321_SIAL

T4321_SIGMA

T8566_SIGMA

tirosina

Tirosina

Tirosina [Spanish]

tyr

Tyr

TYR NH3+ COOH

tyrosine

Tyrosine

TYROSINE

Tyrosine (USP/INN)

Tyrosine (VAN)

Tyrosine [USAN:INN]

Tyrosine Power

Tyrosine, L- (8CI)

Tyrosine, L isomer

Tyrosine, L-isomer

Tyrosinum

Tyrosinum [Latin]

UNII-42HK56048U

W373605_ALDRICH

Y

13

Protein Kinase Inhibitors

Phase 2

14

Immunosuppressive Agents

Phase 2

15

Angiogenesis Inhibitors

Phase 2

16

Immunologic Factors

Phase 2

17

Anti-Infective Agents

Phase 2

18

Anti-Bacterial Agents

Phase 2

Interventional clinical trials:

#	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 3 Study to Compare Oral Masitinib to Placebo in Treatment of Patients With Smouldering or Indolent Severe Systemic Mastocytosis, Unresponsive to Optimal Symptomatic Treatment	Recruiting	NCT04333108	Phase 3	Masitinib
3	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,
4	Phase II Study of Thalidomide in Mastocytosis	Completed	NCT00769587	Phase 2	thalidomide
5	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
6	Mast Cell Connect: A Registry for Patients With Mastocytosis	Completed	NCT02620254

Search NIH Clinical Center for Smoldering Systemic Mastocytosis

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Genetic Tests for Smoldering Systemic Mastocytosis

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Anatomical Context for Smoldering Systemic Mastocytosis

MalaCards organs/tissues related to Smoldering Systemic Mastocytosis:

⁴⁰

Bone Marrow

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Publications for Smoldering Systemic Mastocytosis

Articles related to Smoldering Systemic Mastocytosis:

#	Title	Authors	PMID	Year
1	Systemic mastocytosis associated with myelodysplastic/myeloproliferative neoplasms with ring sideroblasts and thrombocytosis: Report of three cases. ⁶¹	Mimiola E...Zanotti R	31523839	2019
2	Novel approaches to treating advanced systemic mastocytosis. ⁶¹	Gilreath JA...Deininger MW	31372066	2019
3	Diagnostic value of tryptase in anaphylaxis and mastocytosis. ⁶¹	Schwartz LB	16931288	2006
4	Bone marrow mast cell immunophenotyping in adults with mast cell disease: a prospective study of 33 patients. ⁶¹	Pardanani A...Tefferi A	15203275	2004
5	["Smoldering systemic mastocytosis. "Successful therapy with cladribine]. ⁶¹	Schleyer V...Szeimies RM	15241520	2004

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Genes for Smoldering Systemic Mastocytosis

Genes related to Smoldering Systemic Mastocytosis (1 elite genes):

- Elite gene

- Cancer Census gene in COSMIC

#	Symbol	Description	Category	Score	Evidence	PubMed IDs
1	KIT	KIT Proto-Oncogene, Receptor Tyrosine Kinase	Protein Coding	350	Causative somatic mutation ⁵⁸

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Variations for Smoldering Systemic Mastocytosis

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Expression for Smoldering Systemic Mastocytosis

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

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Pathways for Smoldering Systemic Mastocytosis

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GO Terms for Smoldering Systemic Mastocytosis

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Sources for Smoldering Systemic Mastocytosis

¹ BioSystems

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