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, 52Orphanet, 66UMLS, 28ICD10, 37MeSH, 43NCIt, 29ICD10 via Orphanet, 67UMLS via Orphanet, 60SNOMED-CT
See all MalaCards sources

Aliases & Descriptions for Aggressive Systemic Mastocytosis:

Name: Aggressive Systemic Mastocytosis 11 13 52 66
Lymphadenopathic Mastocytosis with Eosinophilia 11
 
Asm 11

Characteristics:

Orphanet epidemiological data:

52
aggressive systemic mastocytosis:
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe); Age of onset: Adult

Classifications:



External Ids:

Disease Ontology11 DOID:4798
ICD1028 C96.2
MeSH37 D034721
NCIt43 C9285
Orphanet52 ORPHA98850
ICD10 via Orphanet29 C96.2
UMLS via Orphanet67 C1112486

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 44)
idRelated DiseaseScoreTop Affiliating Genes
1systemic mastocytosis11.0
2mastocytosis10.5
3malignant biphasic mesothelioma10.5IFNA2, KIT
4melanoma10.4IFNA2, KIT
5hypereosinophilic syndrome, idiopathic, resistant to imatinib10.4IFNA2, KIT
6filamentary keratitis10.4KIT, KITLG
7corneal granular dystrophy10.4KIT, KITLG
8diffuse cutaneous mastocytosis10.4KIT, KITLG
9pericoronitis10.4KIT, KITLG
10esophagus leiomyosarcoma10.4IFNA2, KIT
11autosomal recessive non-syndromic intellectual disability10.4KIT, KITLG
12cutaneous solitary mastocytoma10.3KIT, KITLG
13nasopharynx carcinoma10.3KIT, KITLG
14asrar facharzt haque syndrome10.3KIT, KITLG
15fallopian tube serous papilloma10.3KITLG, PTPN11
16dentinogenesis imperfecta, shields type ii10.2KIT, KITLG
17multiple spinal canal and spinal cord meningioma10.2KIT, KITLG
18restrictive cardiomyopathy10.2KIT, KITLG
19loeffler syndrome10.1KIT, KITLG
20mastroiacovo de rosa satta syndrome10.1IFNA2, KIT, KITLG
21temporal lobe epilepsy10.1IFNA2, KIT, KITLG
22homocystinuria10.0KIT, KITLG
23duodenal obstruction10.0IFNA2, KIT, KITLG
24thrombocythemia 310.0IFNA2, KIT, KITLG
25peroxisome biogenesis disorder 7a10.0IFNA2, KIT, KITLG
26single ventricular heart10.0KITLG, PTPN11
27hepatitis10.0
28leukemia10.0
29mast-cell leukemia10.0
30prostate cancer, hereditary, x-linked 110.0KIT, KITLG
31leukemia, acute myeloid9.9KIT, KITLG, PTPN11
32dubin-johnson syndrome9.8IFNA2, KIT
33glomerulonephritis9.8
34lymphoma9.8
35myeloma9.8
36protein-losing enteropathy9.8
37sarcoma9.8
38cholestasis9.8
39sclerosing cholangitis9.8
40histiocytosis9.8
41cholangitis9.8
42eosinophilia9.8
43myelodysplastic syndrome9.8KIT, KITLG, PTPN11
44movement disease7.2H19, 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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Symptoms:

 52 (show all 40)
  • xerostomia
  • depression
  • anxiety
  • osteoporosis
  • hyperhidrosis
  • pruritus
  • cirrhosis
  • cardiac arrest
  • splenomegaly
  • weight loss
  • pancytopenia
  • eosinophilia
  • leukopenia
  • anemia
  • diarrhea
  • nausea and vomiting
  • malabsorption
  • abdominal pain
  • chronic diarrhea
  • anorexia
  • dyspnea
  • asthma
  • pulmonary infiltrates
  • hepatomegaly
  • acute leukemia
  • hypotension
  • bone pain
  • lymphoma
  • lymphadenopathy
  • recurrent fractures
  • arthralgia
  • myeloproliferative disorder
  • chronic leukemia
  • abnormality of bone marrow cell morphology
  • increased bone mineral density
  • arrhythmia
  • fatigue
  • sarcoma
  • mastocytosis
  • anaphylactic shock

HPO human phenotypes related to Aggressive Systemic Mastocytosis:

(show all 36)
id Description Frequency HPO Source Accession
1 hyperhidrosis hallmark (90%) HP:0000975
2 urticaria hallmark (90%) HP:0001025
3 hypermelanotic macule hallmark (90%) HP:0001034
4 splenomegaly hallmark (90%) HP:0001744
5 abnormality of eosinophils hallmark (90%) HP:0001879
6 nausea and vomiting hallmark (90%) HP:0002017
7 abdominal pain hallmark (90%) HP:0002027
8 hepatomegaly hallmark (90%) HP:0002240
9 bone pain hallmark (90%) HP:0002653
10 impaired temperature sensation hallmark (90%) HP:0010829
11 mastocytosis hallmark (90%) HP:0100495
12 behavioral abnormality typical (50%) HP:0000708
13 pruritus typical (50%) HP:0000989
14 sudden cardiac death typical (50%) HP:0001645
15 leukopenia typical (50%) HP:0001882
16 anemia typical (50%) HP:0001903
17 diarrhea typical (50%) HP:0002014
18 malabsorption typical (50%) HP:0002024
19 hypotension typical (50%) HP:0002615
20 arthralgia typical (50%) HP:0002829
21 abnormality of the gastric mucosa typical (50%) HP:0004295
22 reduced bone mineral density typical (50%) HP:0004349
23 xerostomia occasional (7.5%) HP:0000217
24 weight loss occasional (7.5%) HP:0001824
25 anorexia occasional (7.5%) HP:0002039
26 respiratory insufficiency occasional (7.5%) HP:0002093
27 asthma occasional (7.5%) HP:0002099
28 pulmonary infiltrates occasional (7.5%) HP:0002113
29 acute leukemia occasional (7.5%) HP:0002488
30 lymphoma occasional (7.5%) HP:0002665
31 lymphadenopathy occasional (7.5%) HP:0002716
32 recurrent fractures occasional (7.5%) HP:0002757
33 bone marrow hypocellularity occasional (7.5%) HP:0005528
34 increased bone mineral density occasional (7.5%) HP:0011001
35 sarcoma occasional (7.5%) HP:0100242
36 telangiectasia of the skin occasional (7.5%) HP:0100585

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 10)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
ThalidomidePhase 2106050-35-15426
Synonyms:
( inverted question mark)-Thalidomide
(+)-Thalidomide
(+-)-N-(2,6-dioxo-3-Piperidyl)phthalimide
(+-)-Thalidomide
(+/-)-THALIDOMIDE
(?)-Thalidomide
(±)-N-(2,6-dioxo-3-piperidyl)phthalimide
(±)-thalidomide
.alpha.-(N-Phthalimido)glutarimide
.alpha.-N-Phthalylglutaramide
.alpha.-Phthalimidoglutarimide
1,3-dioxo-2-(2,6-dioxopiperidin-3-yl)isoindoline
14088-68-7
2,6-Dioxo-3-phthalimidopiperidine
2,6-dioxo-3-phthalimidopiperidine
3-Phthalimidoglutarimide
50-35-1
731-40-8
AB00052362
AC-917
AC1L1KBZ
AC1Q6FJA
AI3-50606
Algosediv
Asidon 3
Asmadion
Asmaval
BPBio1_000159
BRD-A93255169-001-06-9
BRN 0030233
BSPBio_000143
BSPBio_001156
BSPBio_003330
Bio1_000387
Bio1_000876
Bio1_001365
Bio2_000418
Bio2_000898
Bonbrain
Bonbrrin
C07910
C13H10N2O4
CCRIS 8148
CHEBI:105028
CHEMBL468
CID5426
CPD000058524
Calmore
Calmorex
Celgene Brand of Thalidomide
Contergan
Corronarobetin
D00754
D013792
DB01041
Distaval
Distaxal
Distoval
DivK1c_000051
E-217
EINECS 200-031-1
ENMD 0995
EU-0101224
Ectiluran
Enterosediv
Gastrinide
Glupan
Glutanon
Grippex
HMS1362J17
HMS1568H05
HMS1792J17
HMS1922E12
HMS1990J17
HMS2090O05
HMS2093G15
HMS500C13
HSDB 3586
Hippuzon
I06-0197
IDI1_000051
IDI1_002173
IN1061
Imida-Lab
Imida-lab
Imidan
Imidan (peyta)
Imidene
Isomin
K 17
K-17
KBio1_000051
KBio2_000496
KBio2_002322
KBio2_003064
KBio2_004890
KBio2_005632
KBio2_007458
KBio3_000911
KBio3_000912
KBio3_002550
KBio3_002802
KBioGR_000496
KBioGR_001474
KBioGR_002322
KBioSS_000496
KBioSS_002324
Kedavon
Kevadon
LS-109463
LS-140
Lopac0_001224
Lulamin
MLS000069353
MolPort-003-665-582
N-(2,6-Dioxo-3-piperidyl)phthalimide
N-(2,6-dioxo-3-piperidyl)phthalimide
N-Phthalimidoglutamic acid imide
N-Phthaloylglutamimide
N-Phthalyl-glutaminsaeure-imid
N-Phthalyl-glutaminsaeure-imid [German]
N-Phthalylglutamic acid imide
NCGC00015989-03
NCGC00015989-13
NCGC00024708-02
 
NCGC00024708-03
NCGC00024708-04
NCGC00024708-05
NCGC00024708-06
NCGC00024708-07
NCGC00024708-08
NCGC00024708-09
NCI60_023904
NCIOpen2_003188
NINDS_000051
NSC 527179
NSC-66847
NSC527179
NSC66847
NSC91729
NSC91730
Neaufatin
Neo
Neosedyn
Neosydyn
Nerosedyn
Neufatin
Neurodyn
Neurosedin
Neurosedym
Neurosedyn
Nevrodyn
Nibrol
Noctosediv
Noxodyn
Pangul
Pantosediv
Poly-Giron
Polygripan
Predni-Sediv
Prestwick0_000192
Prestwick1_000192
Prestwick2_000192
Prestwick3_000192
Prestwick_463
Pro-Bam M
Pro-ban M
Profarmil
Psycholiquid
Psychotablets
Quetimid
Quietoplex
S1193_Selleck
SAM002564245
SMR000058524
SPBio_000893
SPBio_002064
SPECTRUM1503607
ST51039042
Sandormin
Sedalis
Sedalis sedi-lab
Sedimide
Sedin
Sedisperil
Sedoval
Shin-naito S
Shinnibrol
Sleepan
Slipro
Softenil
Softenon
Spectrum2_000707
Spectrum3_001715
Spectrum4_001087
Spectrum5_001791
Synovir
T144_SIGMA
T150_SIGMA
T151_SIGMA
THALIDOMIDE (AIDS INITIATIVE)
Talargan
Talidomida
Talidomida [INN-Spanish]
Talidomide
Talidomide [DCIT]
Talimol
Talismol
Talizer
Telagan
Telargan
Telargean
Tensival
Thaled
Thaled (TN)
Thalidomide
Thalidomide (+ and -)
Thalidomide (JAN/USP/INN)
Thalidomide (soluble form)
Thalidomide Celgene
Thalidomide Pharmion
Thalidomide [USAN:INN:BAN]
Thalidomidum
Thalidomidum [INN-Latin]
Thalidomine USP26
Thalin
Thalinette
Thalomid
Thalomid (TM)
Thalomid (TN)
Thalomid, Thalidomide
Thalomide
Theophilcholine
UNII-4Z8R6ORS6L
UPCMLD-DP139
UPCMLD-DP139:001
Valgis
Valgraine
WLN: T56 BVNVJ C- DT6VMVTJ
WLN: T56 BVNVJ C- DT6VMVTJ -D
WLN: T56 BVNVJ C- DT6VMVTJ -L
Yodomin
alpha-(N-Phthalimido)glutarimide
alpha-N-Phthalylglutaramide
alpha-Phthalimidoglutarimide
alpha-phthalimidoglutarimide
cMAP_000022
thalidomide
α-(N-phthalimido)glutarimide
α-N-phthalylglutaramide
α-phthalimidoglutarimide
2Imatinib MesylatePhase 2588123596
3StaurosporinePhase 24562996-74-1
4tyrosineNutraceuticalPhase 2633
5
SunitinibPhase 1493341031-54-7, 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
6
Mycophenolic acid91924280-93-1446541
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
7
Fludarabine109221679-14-1, 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
8
Mycophenolate mofetil919128794-94-55281078
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
9
Miconazole357322916-47-84189
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
10
Vidarabine47524356-66-932326, 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

Interventional clinical trials:

(show all 15)
idNameStatusNCT IDPhase
1Thalidomide in Treating Patients With Relapsed or Progressive Systemic MastocytosisCompletedNCT00769587Phase 2
2Ibrutinib in Treating Patients With Advanced Systemic MastocytosisRecruitingNCT02415608Phase 2
3Efficacy and Safety of Midostaurin in Patients With Aggressive Systemic Mastocytosis or Mast Cell LeukemiaActive, not recruitingNCT00782067Phase 2
4Phase II Midostaurin in Aggressive Systemic Mastocytosis and Mast Cell LeukemiaActive, not recruitingNCT00233454Phase 2
5Midostaurin in Indolent Systemic MastocytosisActive, not recruitingNCT01920204Phase 2
6Imatinib Mesylate (Gleevec) in the Treatment of Systemic SclerosisActive, not recruitingNCT00555581Phase 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
11Fludarabine and Total-Body Irradiation Followed By Donor Stem Cell Transplant and Cyclosporine and Mycophenolate Mofetil in Treating HIV-Positive Patients With or Without CancerCompletedNCT00112593
12Patient-Reported Outcome Questionnaire for Systemic MastocytosisRecruitingNCT02380222
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:

34
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:

39
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
6ovarian follicle developmentGO:000154110.0KIT, KITLG
7glycosphingolipid metabolic processGO:000668710.0KIT, SMPD1
8positive regulation of MAP kinase activityGO:004340610.0KIT, 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
25GTR
26HGMD
27HMDB
28ICD10
29ICD10 via Orphanet
30ICD9CM
31IUPHAR
32KEGG
35MedGen
37MeSH
38MESH via Orphanet
39MGI
42NCI
43NCIt
44NDF-RT
47NINDS
48Novoseek
50OMIM
51OMIM via Orphanet
55PubMed
56QIAGEN
61SNOMED-CT via Orphanet
65Tumor Gene Family of Databases
66UMLS
67UMLS via Orphanet