MCID: AGG002
MIFTS: 42

Aggressive Systemic Mastocytosis malady

Categories: Blood diseases, Rare diseases, Immune diseases

Aliases & Classifications for Aggressive Systemic Mastocytosis

About this section
Sources:
11Disease Ontology, 13DISEASES, 29ICD10, 30ICD10 via Orphanet, 38MeSH, 44NCIt, 53Orphanet, 61SNOMED-CT, 67UMLS, 68UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Aggressive Systemic Mastocytosis:

Name: Aggressive Systemic Mastocytosis 11 53 13 67
Lymphadenopathic Mastocytosis with Eosinophilia 11
 
Asm 11

Characteristics:

Orphanet epidemiological data:

53
aggressive systemic mastocytosis:
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe); Age of onset: Adult

Classifications:



External Ids:

Disease Ontology11 DOID:4798
ICD1029 C96.2
MeSH38 D034721
NCIt44 C9285
Orphanet53 ORPHA98850
UMLS via Orphanet68 C1112486
ICD10 via Orphanet30 C96.2

Summaries for Aggressive Systemic Mastocytosis

About this section
MalaCards based summary: Aggressive Systemic Mastocytosis, also known as lymphadenopathic mastocytosis with eosinophilia, is related to systemic mastocytosis and mastocytosis, and has symptoms including hyperhidrosis, urticaria and hypermelanotic macule. An important gene associated with Aggressive Systemic Mastocytosis is KIT (KIT Proto-Oncogene Receptor Tyrosine Kinase), and among its related pathways are Melanocyte Development and Pigmentation and Regulation of KIT signaling. Affiliated tissues include bone, bone marrow and myeloid, and related mouse phenotype reproductive system.

Related Diseases for Aggressive Systemic Mastocytosis

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

(show all 45)
idRelated DiseaseScoreTop Affiliating Genes
1systemic mastocytosis10.4
2mastocytosis10.4
3malignant biphasic mesothelioma10.4IFNA2, KIT
4filamentary keratitis10.3KIT, KITLG
5corneal granular dystrophy10.3KIT, KITLG
6diffuse cutaneous mastocytosis10.3KIT, KITLG
7pericoronitis10.3KIT, KITLG
8autosomal recessive non-syndromic intellectual disability10.3KIT, KITLG
9hypereosinophilic syndrome, idiopathic, resistant to imatinib10.3IFNA2, KIT
10cutaneous solitary mastocytoma10.3KIT, KITLG
11esophagus leiomyosarcoma10.3IFNA2, KIT
12asrar facharzt haque syndrome10.3KIT, KITLG
13dentinogenesis imperfecta, shields type ii10.2KIT, KITLG
14fallopian tube serous papilloma10.2KITLG, PTPN11
15multiple spinal canal and spinal cord meningioma10.2KIT, KITLG
16restrictive cardiomyopathy10.1KIT, KITLG
17dubin-johnson syndrome10.1IFNA2, KIT
18loeffler syndrome10.1KIT, KITLG
19mastroiacovo de rosa satta syndrome10.1IFNA2, KIT, KITLG
20temporal lobe epilepsy10.0IFNA2, KIT, KITLG
21duodenal obstruction10.0IFNA2, KIT, KITLG
22thrombocythemia 310.0IFNA2, KIT, KITLG
23peroxisome biogenesis disorder 7a10.0IFNA2, KIT, KITLG
24single ventricular heart9.9KITLG, PTPN11
25hepatitis9.8
26leukemia9.8
27mast-cell leukemia9.8
28leukemia, acute myeloid9.8KIT, KITLG, PTPN11
29prostate cancer, hereditary, x-linked 19.8KIT, KITLG
30myelodysplastic syndrome9.8KIT, KITLG, PTPN11
31pick disease9.8
32niemann-pick disease9.8
33skin disease9.8
34skin atrophy9.8
35acid sphingomyelinase deficiency9.8
36glomerulonephritis9.7
37lymphoma9.7
38protein-losing enteropathy9.7
39sarcoma9.7
40cholestasis9.7
41sclerosing cholangitis9.7
42histiocytosis9.7
43cholangitis9.7
44eosinophilia9.7
45movement disease7.5H19, IFNA2, KIT, KITLG, PTPN11, SMPD1

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



Diseases related to aggressive systemic mastocytosis

Symptoms for Aggressive Systemic Mastocytosis

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Human phenotypes related to Aggressive Systemic Mastocytosis:

 63 53 (show all 51)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hyperhidrosis63 53 hallmark (90%) Very frequent (99-80%) HP:0000975
2 urticaria63 hallmark (90%) HP:0001025
3 hypermelanotic macule63 hallmark (90%) HP:0001034
4 splenomegaly63 53 hallmark (90%) Very frequent (99-80%) HP:0001744
5 abnormality of eosinophils63 hallmark (90%) HP:0001879
6 nausea and vomiting63 53 hallmark (90%) Very frequent (99-80%) HP:0002017
7 abdominal pain63 53 hallmark (90%) Frequent (79-30%) HP:0002027
8 hepatomegaly63 53 hallmark (90%) Frequent (79-30%) HP:0002240
9 bone pain63 53 hallmark (90%) Very frequent (99-80%) HP:0002653
10 impaired temperature sensation63 hallmark (90%) HP:0010829
11 mastocytosis63 53 hallmark (90%) Very frequent (99-80%) HP:0100495
12 behavioral abnormality63 typical (50%) HP:0000708
13 pruritus63 53 typical (50%) Frequent (79-30%) HP:0000989
14 sudden cardiac death63 typical (50%) HP:0001645
15 leukopenia63 53 typical (50%) Frequent (79-30%) HP:0001882
16 anemia63 53 typical (50%) Frequent (79-30%) HP:0001903
17 diarrhea63 53 typical (50%) Frequent (79-30%) HP:0002014
18 malabsorption63 53 typical (50%) Frequent (79-30%) HP:0002024
19 hypotension63 53 typical (50%) Frequent (79-30%) HP:0002615
20 arthralgia63 53 typical (50%) Frequent (79-30%) HP:0002829
21 abnormality of the gastric mucosa63 typical (50%) HP:0004295
22 reduced bone mineral density63 typical (50%) HP:0004349
23 xerostomia63 53 occasional (7.5%) Occasional (29-5%) HP:0000217
24 weight loss63 53 occasional (7.5%) Occasional (29-5%) HP:0001824
25 anorexia63 53 occasional (7.5%) Occasional (29-5%) HP:0002039
26 respiratory insufficiency63 occasional (7.5%) HP:0002093
27 asthma63 53 occasional (7.5%) Occasional (29-5%) HP:0002099
28 pulmonary infiltrates63 53 occasional (7.5%) Occasional (29-5%) HP:0002113
29 acute leukemia63 53 occasional (7.5%) Occasional (29-5%) HP:0002488
30 lymphoma63 53 occasional (7.5%) Occasional (29-5%) HP:0002665
31 lymphadenopathy63 53 occasional (7.5%) Occasional (29-5%) HP:0002716
32 recurrent fractures63 53 occasional (7.5%) Occasional (29-5%) HP:0002757
33 bone marrow hypocellularity63 occasional (7.5%) HP:0005528
34 increased bone mineral density63 53 occasional (7.5%) Occasional (29-5%) HP:0011001
35 sarcoma63 53 occasional (7.5%) Occasional (29-5%) HP:0100242
36 telangiectasia of the skin63 occasional (7.5%) HP:0100585
37 depression53 Frequent (79-30%)
38 anxiety53 Frequent (79-30%)
39 osteoporosis53 Frequent (79-30%)
40 cirrhosis53 Occasional (29-5%)
41 cardiac arrest53 Frequent (79-30%)
42 pancytopenia53 Occasional (29-5%)
43 eosinophilia53 Very frequent (99-80%)
44 chronic diarrhea53 Frequent (79-30%)
45 dyspnea53 Occasional (29-5%)
46 myeloproliferative disorder53 Occasional (29-5%)
47 chronic leukemia53 Occasional (29-5%)
48 abnormality of bone marrow cell morphology53 Very frequent (99-80%)
49 arrhythmia53 Frequent (79-30%)
50 fatigue53 Very frequent (99-80%)
51 anaphylactic shock53 Frequent (79-30%)

UMLS symptoms related to Aggressive Systemic Mastocytosis:


hepatomegaly

Drugs & Therapeutics for Aggressive Systemic Mastocytosis

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

(show all 30)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1StaurosporineexperimentalPhase 24562996-74-1
24'-N-benzoylstaurosporinePhase 223
3Imatinib MesylatePhase 2594123596
4Protein Kinase InhibitorsPhase 23612
5Specific substance maruyamaPhase 214
6Anti-Infective AgentsPhase 221402
7Anti-Bacterial AgentsPhase 210884
8Immunosuppressive AgentsPhase 212770
9Angiogenesis Modulating AgentsPhase 2, Phase 14072
10Angiogenesis InhibitorsPhase 2, Phase 14143
11tyrosineNutraceuticalPhase 2659
12
Sunitinibapproved, investigationalPhase 1500341031-54-7, 557795-19-45329102
Synonyms:
(2S)-2-hydroxybutanedioic acid
1H-Pyrrole-3-carboxamide, N-(2-(diethylamino)ethyl)-5-((Z)-(5-fluoro-1,2-dihydro-2-oxo-3H-indol-3-ylidene)methyl)-2,4-dimethyl-, (2S)-hydroxybutanedioate (1:1)
1H-Pyrrole-3-carboxamide, N-(2-(diethylamino)ethyl)-5-((Z)-(5-fluoro-1,2-dihydro-2-oxo-3H-indol-3-ylidene)methyl)-2,4-dimethyl-, (2S)-hydroxybutanedioate (1:1)
326914-13-0
341031-54-7
5-(5-FLUORO-2-OXO-1,2-DIHYDRO-INDOL-3-YLIDENEMETHYL)-2,4-DIMETHYL-1H-PYRROLE-3-CARBOXYLIC ACID (2-DIETHYLAMINO-ETHYL)-AMIDE
557795-19-4
AC1NS62J
AC1O5CMQ
AKOS005145765
Butanedioic acid, hydroxy-, (2S)-, compd. with N-(2-(diethylamino)ethyl)-5-((Z)-(5-fluoro-1,2-dihydro-2-oxo-3H-indol-3-ylidene)methyl)-2,4-dimethyl-1H-pyrrole-3-carboxamide (1:1)
CHEBI:38940
CHEBI:550864
CHEMBL1567
CHEMBL535
CID5329102
CID6456015
D06402
D08552
DB01268
DB07417
EN002687
FT-0083555
FT-0083556
I01-1229
K00588a
KS-5022
LS-186078
LS-187023
LS-187648
MolPort-003-986-763
N-(2-(Diethylamino)ethyl)-5-((Z)-(5-fluoro-1,2-dihydro-2-oxo-3H-indol-3-ylidene)methyl)-2,4-dimethyl-1H-pyrrole-3-carboxamide (2S)-hydroxybutanedioate
N-(2-diethylaminoethyl)-5-[(Z)-(5-fluoro-2-oxo-1H-indol-3-ylidene)methyl]-2,4-dimethyl-1H-pyrrole-3-carboxamide
N-[2-(diethylamino)ethyl]-5-[(Z)-(5-fluoro-2-oxo-1,2-dihydro-3H-indol-3-ylidene)methyl]-2,4-dimethyl-1H-pyrrole-3-carboxamide
NCGC00164631-01
 
NSC736511
NSC750690
PDGF TK antagonist
PHA-290940AD
PNU-290940AD
S1042_Selleck
ST51053712
SU 011248
SU 11248
SU-010398
SU-011248 L-malate salt
SU-11248
SU-11248 L-malate salt
SU-11248J
SU-12662
SU010398
SU011248
SU011248 L-malate salt
SU11248
Su-011248
Sunitanib
Sunitinib
Sunitinib (INN)
Sunitinib (free base)
Sunitinib malate
Sunitinib malate (JAN/USAN)
Sunitinib malate [USAN]
Sunitinibum
Sutent
Sutent (TN)
Sutent, SU-11248
TL8002546
UNII-LVX8N1UT73
UNII-V99T50803M
sunitinib
sunitinibum
13
Mycophenolate mofetilapproved, investigational939128794-94-55281078
Synonyms:
115007-34-6
128794-94-5
140401-05-4
2-Morpholinoethyl (4E)-6-(4-hydroxy-6-methoxy-7-methyl-3-oxo-5-phthalanyl)-4-methyl-4-hexenoate
2-Morpholinoethyl (E)-6-(4-hydroxy-6-methoxy-7-methyl-3-oxo-5-phthalanyl)-4-methyl-4-hexenoate
2-Morpholinoethyl (e)-6-(4-hydroxy-6-methoxy-7-methyl-3-oxo-5-phthalanyl)-4-methyl-4-hexenoate
2-morpholin-4-ylethyl (4E)-6-[4-hydroxy-7-methyl-6-(methyloxy)-3-oxo-1,3-dihydro-2-benzofuran-5-yl]-4-methylhex-4-enoate
2-morpholin-4-ylethyl (E)-6-(4-hydroxy-6-methoxy-7-methyl-3-oxo-1H-2-benzofuran-5-yl)-4-methylhex-4-enoate
4-Hexenoic acid, 6-(1,3-dihydro-4-hydroxy-6-methoxy-7-methyl -3-oxo-5-isobenzofuranyl)-4-methyl-, 2-(4-morpholinyl)ethyl ester, (4E)
AC-1562
AC1NQXZW
AC1Q6O6X
AR-1J6939
BB_NC-2566
C07908
C23H31NO7
CHEMBL1456
CID5281078
CellCept
CellCept, RS 61443, TM-MMF, Mycophenolate mofetil
Cellcept
Cellcept (TN)
D00752
DB00688
HMS2090A03
 
HSDB 7436
I01-0898
I06-1947
LS-172272
LS-75572
ME-MPA
MMF
MMF CellCept(TM)
MolPort-000-883-800
Munoloc
Mycophenolate mofetil (JAN/USAN)
Mycophenolic acid morpholinoethyl ester
Mycophenylate mofetil
NCGC00159459-02
NCGC00159459-03
NSC724229
R-99
RS 61443
RS-61443
RS-61443-190
S1501_Selleck
TL8000648
TM-MMF
UNII-9242ECW6R0
ZINC21297660
mycophenolate mofetil
14
Mycophenolic acidapproved93924280-93-1446541
Synonyms:
(e)-6-(4-Hydroxy-6-methoxy-7-methyl-3-oxo-5-phthalanyl)-4-methyl-4-hexenoic acid
Acide mycophenolique
Acido micofenolico
Acidum mycophenolicum
 
Melbex
Micofenolico acido
Mycophenoic acid
Mycophenolate
Mycophenolsäure
Myfortic
15
Cyclosporineapproved, investigational, vet_approved91579217-60-0, 59865-13-35284373, 6435893
Synonyms:
1c5f
1cyn
30024_FLUKA
30024_SIGMA
59865-13-3
79217-60-0
AC1L1EQW
AC1NQXJE
AC1NR4C4
AC1NUQK3
AC1NUZNC
AC1O5KOG
AC1Q2UDG
Ambap59865-13-3
Ambotz59865-13-3
Antibiotic S 7481F1
BMT-ABA-SAR-MLE-VAL-MLE-ALA-ALA-MLE-MLE-MVA
BMT-ABA-SAR-MLE-VAL-MLE-ALA-DAL-MLE-MLE-MVA
BPBio1_000496
BRD-A64290322-001-01-6
BRD-A69815203-001-04-3
BRD-K13533483-001-03-0
BSPBio_000450
BSPBio_001596
BSPBio_003186
C 3662
C05086
C1832_SIGMA
C3662_SIGMA
C62H111N11O12
CB-01-09 MMX
CHEBI:106343
CHEBI:328305
CHEBI:4031
CHEMBL160
CHEMBL386389
CHEMBL532318
CID2909
CID5280754
CID5284373
CID5458585
CID5497195
CID6435893
CSA
CYCLOSPORIN A (SEE ALSO TRANSGENIC MODEL EVALUATION (CYCLOSPORIN A))
CYCLOSPORIN A, USP
Ciclosporin
Ciclosporin (JP15)
Ciclosporina
Ciclosporine
Ciclosporinum
Cipol N
Cipol-N
Consupren
Consupren S
CsA
CsA & IFN.alpha.
CyA
Cyclokat
Cyclosporin
Cyclosporin A
Cyclosporin A & IFN.alpha.
Cyclosporin A Implant
Cyclosporin A, Tolypocladium inflatum
Cyclosporine (USP)
Cyclosporine A
Cyclosporine [USAN]
D00184
DE-076
DivK1c_000871
EU-0100242
Equoral
GNF-Pf-2808
Gengraf
Gengraf (TN)
HMS1569G12
HMS1791P18
HMS1921L20
HMS1989P18
HMS2089A09
HMS2092F06
HMS502L13
Helv Chim Acta 60: 1568 (1977)
 
I06-0379
I06-0966
IDI1_000871
KBio1_000871
KBio2_000780
KBio2_003348
KBio2_005916
KBio3_002686
KBioGR_001898
KBioSS_000780
LMPK14000003
LS-257
LS-58836
Lopac0_000242
MLS000028376
MLS001333756
MLS002153454
MLS002207033
Mitogard
Modusik-A
MolPort-000-760-988
MolPort-005-934-008
MolPort-006-705-994
NCGC00093704-01
NCGC00093704-02
NCGC00093704-03
NCGC00093704-04
NCGC00093704-05
NCGC00093704-06
NCGC00093704-07
NCGC00093704-08
NCGC00164258-01
NCGC00164258-02
NINDS_000871
NSC290193
Neoplanta
Neoral
Neoral (TN)
NeuroSTAT
Nova-22007
OL 27-400
OL-27400
OLO-400
Papilock
Prestwick2_000435
Prestwick3_000435
Prestwick_731
Pulminiq
Ramihyphin A
Restasis
Restasis (TN)
S-Neoral
S1514_Selleck
SDZ-OXL 400
SMR000058578
SPBio_001467
SPECTRUM1502202
ST-603
Sandimmun
Sandimmun Neoral
Sandimmune
Sandimmune (TN)
Sandimmune, Gengraf, Restasis, Atopica, Sangcya, Cyclosporine
Sang-2000
Sang-35
SangCyA
Sangcya
Sigmasporin
Sigmasporin Microoral
Spectrum2_001484
Spectrum3_001593
Spectrum4_001279
Spectrum5_001628
Spectrum_000300
TRANSGENIC MODEL EVALUATION (CYCLOSPORIN A)
Vekacia
Zyclorin
cyclophorine
cyclosporin A
cyclosporine
from Tolypocladium inflatum (Trichoderma polysporin)
nchembio.184-comp6
nchembio.301-comp5
nchembio.342-comp1
16
Fludarabineapproved111321679-14-1, 75607-67-930751
Synonyms:
(2R,3S,4S,5R)-2-(6-amino-2-fluoro-9H-purin-9-yl)-5-(hydroxymethyl)oxolane-3,4-diol
(2R,3S,4S,5R)-2-(6-amino-2-fluoropurin-9-yl)-5-(hydroxymethyl)oxolane-3,4-diol
2-F-ARAA
2-F-ara-A
2-Fluoro Ara-A
2-Fluoro-9-beta-D-arabinofuranosyladenine
2-fluoro ARA-A
21679-14-1
9-beta-D-Arabinofuranosyl-2-fluoroadenine
9-beta-D-arabinofuranosyl-2-fluoro-9H-purin-6-amine
9H-Purin-6-amine, 9-beta-D-arabinofuranosyl-2-fluoro- (9CI)
AC1LCW8I
AC1Q51CF
C10H12FN5O4
CCRIS 3382
CHEMBL1568
CID657237
CPD000058874
D07966
EINECS 244-525-5
F-Ara-A
FAMP
FT-0082766
FaraA
Fludara
Fludara, Fludarabine
 
Fludarabina
Fludarabina [Spanish]
Fludarabine
Fludarabine (INN)
Fludarabine 5'-monophosphate
Fludarabine [INN]
Fludarabine monophosphate
Fludarabine phosphate
Fludarabinum
Fludarabinum [Latin]
Fludura
Fluradosa
Fluradosa (TN)
HSDB 6964
I14-4978
LS-15061
MLS000028687
NSC 118218
NSC 118218H
NSC-118218
S1491_Selleck
SAM002548956
SMR000058874
SQ Fludarabine
UNII-1X9VK9O1SC
UNII-P2K93U8740
ZINC04216238
17
Miconazoleapproved, investigational, vet_approved362422916-47-84189
Synonyms:
(+-)-1-(2,4-Dichloro-beta-((2,4-dichlorobenzyl)oxy)phenethyl)imidazole
1-(2,4-Dichloro-beta-((2,4-dichlorobenzyl)oxy)phenethyl)imidazole
1-(2,4-dichloro-beta-((2,4-dichlorobenzyl)oxy)phenethyl) imidazole
1-[2,4-Dichloro- beta-([2,4-dichloro- benzyl]oxy)phenethyl]imidazole
1-[2-(2,4-Dichloro-benzyloxy)-2-(2,4-dichloro-phenyl)-ethyl]-1H-imidazole
1-[2-(2,4-Dichlorophenyl)-2-[(2,4-dichlorophenyl)methoxy]ethyl]-1H-imidazole
1-[2-(2,4-dichlorobenzyloxy)-2-(2,4-dichlorophenyl)ethyl]-1H-imidazole
1-[2-(2,4-dichlorophenyl)-2-[(2,4-dichlorophenyl)methoxy]ethyl]imidazole
1-[2-(2,4-dichlorophenyl)-2-{[(2,4-dichlorophenyl)methyl]oxy}ethyl]-1H-imidazole
1-{2-[(2,4-dichlorobenzyl)oxy]-2-(2,4-dichlorophenyl)ethyl}-1H-imidazole
22832-87-7 (NITRATE)
22916-47-8
75319-47-0
AB00053500
AC1L1HM1
AKOS001574474
Aflorix(nitrate)
Albistat(nitrate)
Andergin(nitrate)
BPBio1_000279
BRD-A82396632-001-03-0
BRD-A82396632-008-02-7
BRN 0965511
BSPBio_000253
BSPBio_002033
CCRIS 7924
CHEBI:6923
CHEMBL91
CID4189
CPD-4501
Conofite(nitrate)
D00416
DB01110
Dactarin
Daktarin IV
Daktarin iv
DivK1c_000156
EINECS 245-324-5
Epi-Monistat(nitrate)
Femizol-M
Florid(nitrate)
Gyno-Daktar(nitrate)
HMS1568M15
HMS2090B21
I14-14342
IDI1_000156
Imidazole, 1-(2-(2,4-dichlorophenyl)-2-((2,4-dichlorophenyl)methoxy)ethyl)- (9CI)
KBio1_000156
KBio2_001445
KBio2_004013
KBio2_006581
KBio3_001533
KBioGR_000581
KBioSS_001445
LS-78378
Lotrimin AF(nitrate)
MCZ
MJR 1762
MLS002222203
Micantin (nitrate)
Miconasil Nitrate
 
Miconazol
Miconazol [INN-Spanish]
Miconazole
Miconazole (JP15/USP/INN)
Miconazole 3
Miconazole 3 Combination Pack
Miconazole 7 Combination Pack
Miconazole [USAN:BAN:INN:JAN]
Miconazole nitrate salt
Miconazole-7
Miconazolo
Miconazolo [DCIT]
Miconazolum
Miconazolum [INN-Latin]
Micozole
Minostate
MolPort-002-557-553
Monazole 7
Monista (nitrate)
Monistat
Monistat (TN)
Monistat 1 Combination Pack
Monistat 3 Dual-Pak
Monistat 3 Vaginal Ovules
Monistat 5 Tampon
Monistat 7 Dual-Pak
Monistat 7 Vaginal Suppositories
Monistat Dual- PAK
Monistat IV
Monistat iv (TN)
Monistat iv (tn)
Monistat-Derm
NCI60_001353
NCI60_001380
NINDS_000156
NSC 170986
NSC169434
NSC170986
Novo-Miconazole Vaginal Ovules
Oprea1_091955
Prestwick0_000067
Prestwick1_000067
Prestwick2_000067
Prestwick3_000067
Prestwick_335
R 18134
R-14,889
SMR001307249
SPBio_000976
SPBio_002174
STK834405
STOCK1S-93556
Spectrum2_001048
Spectrum3_000507
Spectrum4_000061
Spectrum5_001297
Spectrum_000965
UNII-7NNO0D7S5M
Vusion
Zimycan
imidazole, 1-(2-(2,4-dichlorophenyl)-2-((2,4-dichlorophenyl) methoxy)ethyl)- (9CI)
miconazole
18
Vidarabineapproved51224356-66-932326, 21704
Synonyms:
(+)-Cyclaradine
.beta.-Adenosine
.beta.-D-Adenosine
1odi
2-(6-AMINO-purin-9-yl)-5-hydroxymethyl-tetrahydro-furan-3,4-diol
2946-52-3
2fqy
2gl0
30143-02-3
3080-29-3
3228-71-5
4005-33-8
46946-45-6
46969-16-8
524-69-6
5536-17-4
58-61-7
9-Arabinosyladenine
9-beta-D-Arabinofuranosyl-9H-purin-6-amine
9-beta-D-Arabinofuranosyl-adenine
9-beta-D-Arabinofuranosyladenine
9-beta-D-arabinofuranosyl-adenine
9-β-D-arabinofuranosyl-9H-purin-6-amine
9-β-D-arabinofuranosyladenine
A 9251
A0152
A4036_SIGMA
A4676_SIGMA
A5762_SIGMA
A9251_SIGMA
AC1L18OL
AC1L1U8O
AC1L2IWM
AC1L2SCM
AC1O4WIN
AC1O8PY7
AC1Q1ID3
AC1Q4Y1Z
AC1Q52XU
ADENOSINE, U.S.P.
ADN
AI3-52413
AI3-52821
AR-1H6029
ARA-A NSC 247519
Ade-Rib
Adenine Arabinoside
Adenine arabinoside
Adenine nucleoside
Adenine riboside
Adenine xyloside
Adenocard
Adenocard (TN)
Adenocard, Adenosine
Adenocor
Adenoscan
Adenoscan (TN)
Adenosin
Adenosin [German]
Adenosine (JAN/USP)
Adenosine [USAN:BAN]
Adenosine arabinose
Adenosine-8-14C
Adensoine
Ambap5536-17-4
Ara A
Ara-A
Ara-ATP
Araadenosine
Arabinoside Adenine
Arabinoside adenine
Arabinosyl Adenine
Arabinosyl adenine
Arabinosyl-adenine
Arabinosyladenine
Arasena-A
Armes
Armes (TN)
BB_NC-0565
BPBio1_000898
BRN 0624881
BSPBio_000816
BSPBio_001796
BSPBio_002000
Bio1_000437
Bio1_000926
Bio1_001415
Boniton
C00212
CAS-5536-17-4
CCRIS 2557
CCRIS 3383
CHEBI:136932
CHEBI:16335
CHEBI:45327
CHEMBL1090
CHEMBL20247
CHEMBL477
CI 673
CI-673
CID102198
CID191
CID21704
CID60961
CID6420052
CID6713976
CPD000471872
Caswell No. 010B
D000241
D00045
D06298
DB00640
DivK1c_000191
EINECS 200-389-9
EINECS 217-911-6
EINECS 226-893-9
EU-0100123
FT-0082881
HMS1570I18
HMS1920A13
HMS1921K05
HMS2090F06
HMS2091G13
HMS2092C16
HMS500J13
HSDB 6514
 
I01-1121
IDI1_000191
KBio1_000191
KBio2_002418
KBio2_004986
KBio2_007554
KBio3_001296
KBio3_001500
KBioGR_001224
KBioSS_002424
L000094
LS-15059
LS-15085
Lopac0_000123
MEDR-640
MLS000069638
MLS000699527
MLS001066352
MLS001333133
MLS001333134
MLS002153227
MLS002153992
MolPort-001-785-903
MolPort-001-838-229
MolPort-003-666-308
Myocol
NCGC00016656-01
NCGC00023673-03
NCGC00023673-04
NCGC00023673-05
NCGC00023673-06
NCGC00023673-07
NCGC00094579-01
NCGC00094579-02
NCGC00094579-03
NCGC00094579-04
NCGC00178869-01
NCGC00179417-01
NCI60_003823
NCI60_037192
NCIOpen2_003303
NCIOpen2_005376
NINDS_000191
NSC 247519
NSC 404241
NSC 627048
NSC 7359
NSC 7652
NSC-404241
NSC247519
NSC404241
NSC627048
NSC70422
NSC7359
NSC7652
NSC80832
NSC87676
NSC91041
Nucleocardyl
PDSP1_001036
PDSP2_001020
Pallacor
Polyadenosine
Polyriboadenosine
Prestwick0_000768
Prestwick1_000768
Prestwick2_000768
Prestwick3_000768
Prestwick_983
RAB
S1647_Selleck
S1784_Selleck
SAM002564191
SMP1_000312
SMR000058216
SMR000225041
SMR000471872
SMR001233326
SPBio_001194
SPBio_001491
SPBio_002755
SPECTRUM1500107
SPECTRUM1500609
SR 96225
SR-96225
STK361815
SUN-Y4001
Sandesin
Spectrum2_001257
Spectrum2_001336
Spectrum3_000288
Spectrum3_000580
Spectrum4_000782
Spectrum5_001429
Spectrum_001894
Spongoadenosine
TL8003749
UNII-3XQD2MEW34
UNII-K72T3FS567
USAF CB-10
V0098
VIRDARABINE
Vidarabin
Vidarabina
Vidarabina [DCIT]
Vidarabine
Vidarabine (JAN)
Vidarabine anhydrous
Vidarabinum
Vira ATM
Vira-A
Vira-A, Vidarabine
XA
Xylosyl A
Xylosyladenine
ZINC00970363
ZINC02169830
adenine-D-ribose
adenosine
alpha-Ara A
beta-Adenosine
beta-Ara A
beta-D-Adenosine
bmse000061
nchembio.143-comp9
nchembio.186-comp109
nchembio.64-comp4
nchembio706-5
19Vidarabine Phosphate124
20Antimetabolites11774
21Dermatologic Agents5674
22Antibiotics, Antitubercular6972
23Antimetabolites, Antineoplastic7171
24Antifungal Agents3615
25Antirheumatic Agents10627
26Calcineurin Inhibitors1597
27Immunoglobulins6045
28Antibodies, Monoclonal3795
29Antibodies6045
30Antiviral Agents9732

Interventional clinical trials:

(show all 15)
idNameStatusNCT IDPhase
1Midostaurin in Indolent Systemic MastocytosisUnknown statusNCT01920204Phase 2
2Imatinib Mesylate (Gleevec) in the Treatment of Systemic SclerosisUnknown statusNCT00555581Phase 2
3Thalidomide in Treating Patients With Relapsed or Progressive Systemic MastocytosisCompletedNCT00769587Phase 2
4Ibrutinib in Treating Patients With Advanced Systemic MastocytosisRecruitingNCT02415608Phase 2
5Efficacy and Safety of Midostaurin in Patients With Aggressive Systemic Mastocytosis or Mast Cell LeukemiaActive, not recruitingNCT00782067Phase 2
6Phase II Midostaurin in Aggressive Systemic Mastocytosis and Mast Cell LeukemiaActive, not recruitingNCT00233454Phase 2
7Sunitinib Malate in Treating HIV-Positive Patients With Cancer Receiving Antiretroviral TherapyCompletedNCT00890747Phase 1
8MK2206 in Treating Younger Patients With Recurrent or Refractory Solid Tumors or LeukemiaCompletedNCT01231919Phase 1
9A Safety, Tolerability and PK Study of DCC-2618 in Patients With Advanced MalignanciesRecruitingNCT02571036Phase 1
10Study of BLU-285 in Patients With Advanced Systemic Mastocytosis (advSM) and Relapsed or Refractory Myeloid MalignanciesRecruitingNCT02561988Phase 1
11Patient-Reported Outcome Questionnaire for Systemic MastocytosisCompletedNCT02380222
12Fludarabine and Total-Body Irradiation Followed By Donor Stem Cell Transplant and Cyclosporine and Mycophenolate Mofetil in Treating HIV-Positive Patients With or Without CancerCompletedNCT00112593
13Brentuximab Vedotin in Treating Patients With Advanced Systemic Mastocytosis or Mast Cell LeukemiaRecruitingNCT01807598
14Mast Cell Connect: A Registry for Patients With MastocytosisRecruitingNCT02620254
15Fludarabine Phosphate and Total-Body Radiation Followed by Donor Peripheral Blood Stem Cell Transplant and Immunosuppression in Treating Patients With Hematologic MalignanciesActive, not recruitingNCT00014235

Search NIH Clinical Center for Aggressive Systemic Mastocytosis

Genetic Tests for Aggressive Systemic Mastocytosis

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Anatomical Context for Aggressive Systemic Mastocytosis

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MalaCards organs/tissues related to Aggressive Systemic Mastocytosis:

35
Bone, Bone marrow, Myeloid, Skin, Ovary

Animal Models for Aggressive Systemic Mastocytosis or affiliated genes

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MGI Mouse Phenotypes related to Aggressive Systemic Mastocytosis:

40
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053898.3KIT, KITLG, PTPN11, SMPD1, UBE2B

Publications for Aggressive Systemic Mastocytosis

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Articles related to Aggressive Systemic Mastocytosis:

(show all 27)
idTitleAuthorsYear
1
Aggressive Systemic Mastocytosis mimicking lymphoma: Description of an unusual presentation and review of the literature on current management strategies. (24707942)
2014
2
An unusual case of aggressive systemic mastocytosis mimicking hepatic cirrhosis. (25009756)
2014
3
Oncogenic KIT-induced aggressive systemic mastocytosis requires SHP2/PTPN11 phosphatase for disease progression in mice. (25026279)
2014
4
A transgenic zebrafish model expressing KIT-D816V recapitulates features of aggressive systemic mastocytosis. (24989799)
2014
5
Eosinophilia and bone lesion as clinical manifestations of aggressive systemic mastocytosis. (24369222)
2013
6
An unusual case of aggressive systemic mastocytosis with associated refractory plasma cell myeloma. (23009979)
2012
7
Endoscopic ultrasound-guided fine needle aspiration: a powerful modality in the diagnosis of aggressive systemic mastocytosis. (20497208)
2011
8
Aggressive systemic mastocytosis associated with mesangioproliferative glomerulonephritis. (21196717)
2011
9
Imatinib mesylate for aggressive systemic mastocytosis with long bone osteolysis. (21679828)
2011
10
Aberrant expression of CD30 in aggressive systemic mastocytosis and mast cell leukemia: a differential diagnosis to consider in aggressive hematopoietic CD30-positive neoplasms. (21261503)
2011
11
Treatment of aggressive systemic mastocytosis with daclizumab. (20038223)
2010
12
Aggressive systemic mastocytosis with Charcot-Leyden crystals-associated crystal storing histiocytosis in bone marrow. (20025488)
2010
13
Imatinib mesylate responsiveness in aggressive systemic mastocytosis: novel association with a platelet derived growth factor receptor beta mutation. (17133421)
2007
14
Aggressive systemic mastocytosis complicated by protein-losing enteropathy. (16843741)
2007
15
Juxtamembrane-type c-kit gene mutation found in aggressive systemic mastocytosis induces imatinib-resistant constitutive KIT activation. (17259998)
2007
16
Aggressive systemic mastocytosis after germ cell tumor of the ovary: C-KIT mutation documentation in both disease states. (17551405)
2007
17
Aggressive systemic mastocytosis with sarcoma-like growth in the skeleton, leukemic progression, and partial loss of mast cell differentiation antigens. (18055976)
2007
18
Aggressive systemic mastocytosis presenting with hepatic cholestasis. (17873616)
2007
19
Establishment of a murine model of aggressive systemic mastocytosis/mast cell leukemia. (16543062)
2006
20
Aggressive systemic mastocytosis: a case report and brief review of the literature. (15794480)
2005
21
Aggressive systemic mastocytosis: is there a role for trisomy 8? (15725484)
2005
22
Aggressive systemic mastocytosis mimicking sclerosing cholangitis. (15377487)
2004
23
Response to therapy with interferon alpha-2b and prednisolone in aggressive systemic mastocytosis: report of five cases and review of the literature. (14687620)
2004
24
Inefficacy of imatinib-mesylate in sporadic, aggressive systemic mastocytosis. (15109544)
2004
25
Aggressive systemic mastocytosis. (12639621)
2003
26
Response to cyclosporin and low-dose methylprednisolone in aggressive systemic mastocytosis. (10329843)
1999
27
The presence of membrane-bound stem cell factor on highly immature nonmetachromatic mast cells in the peripheral blood of a patient with aggressive systemic mastocytosis. (8876560)
1996

Variations for Aggressive Systemic Mastocytosis

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Expression for genes affiliated with Aggressive Systemic Mastocytosis

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

Pathways for genes affiliated with Aggressive Systemic Mastocytosis

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GO Terms for genes affiliated with Aggressive Systemic Mastocytosis

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

(show all 12)
idNameGO IDScoreTop Affiliating Genes
1embryonic hemopoiesisGO:003516210.3KIT, KITLG
2ectopic germ cell programmed cell deathGO:003523410.3KIT, KITLG
3megakaryocyte developmentGO:003585510.2KIT, PTPN11
4positive regulation of tyrosine phosphorylation of Stat3 proteinGO:004251710.1IFNA2, KIT
5regulation of type I interferon-mediated signaling pathwayGO:006033810.1IFNA2, PTPN11
6positive regulation of MAP kinase activityGO:004340610.0KIT, KITLG
7glycosphingolipid metabolic processGO:000668710.0KIT, SMPD1
8ovarian follicle developmentGO:000154110.0KIT, KITLG
9phosphatidylinositol phosphorylationGO:00468549.8KIT, KITLG, PTPN11
10regulation of phosphatidylinositol 3-kinase signalingGO:00140669.8KIT, KITLG, PTPN11
11phosphatidylinositol-mediated signalingGO:00480159.7KIT, KITLG, PTPN11
12spermatogenesisGO:00072838.8KIT, TNP1, UBE2B

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

idNameGO IDScoreTop Affiliating Genes
1phosphatidylinositol-4,5-bisphosphate 3-kinase activityGO:00469349.3KIT, KITLG, PTPN11

Sources for Aggressive Systemic Mastocytosis

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
26GTR
27HGMD
28HMDB
29ICD10
30ICD10 via Orphanet
31ICD9CM
32IUPHAR
33KEGG
36MedGen
38MeSH
39MESH via Orphanet
40MGI
43NCI
44NCIt
45NDF-RT
48NINDS
49Novoseek
51OMIM
52OMIM via Orphanet
56PubMed
57QIAGEN
62SNOMED-CT via Orphanet
66Tumor Gene Family of Databases
67UMLS
68UMLS via Orphanet