General Information of Disease (ID: DISFKGJU)

Disease Name Idiopathic thrombocytopenic purpura
Synonyms
immune thrombocytopenic purpura; AITP; idiopathic purpura; thrombocytopenic purpura autoimmune; ideopath thrombocytopenic pur; Werlhof's disease; primary thrombocytopenic purpura; idiopathic thrombocytopenia purpura; immune thrombocytopenia; autoimmune thrombocytopenic purpura; idiopathic thrombocytopenia; ITP; idiopathic thrombocytopenic purpura; thrombocytopenic purpura, autoimmune
Disease Class 3B64: Thrombocytopenia
Definition An autoimmune disorder in which the number of circulating platelets is reduced due to their antibody-mediated destruction. ITP is a diagnosis of exclusion and is heterogeneous in origin.
Disease Hierarchy
DISZI5Z8: Thrombocytopenic purpura
DIS5GLM9: Primary thrombocytopenia
DIS1DL2M: Inherited blood coagulation disorder
DISANU9Q: Inherited thrombocytopenia
DISNF0OI: Autoimmune thrombocytopenia
DISIUNXT: Inherited bleeding disorder, platelet-type
DISFKGJU: Idiopathic thrombocytopenic purpura
ICD Code
ICD-11
ICD-11: 3B64.10
ICD-10
ICD-10: D69, D69.3
Disease Identifiers
MONDO ID
MONDO_0008558
MESH ID
D016553
UMLS CUI
C0398650
OMIM ID
188030
MedGen ID
584986
Orphanet ID
3002

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)
This Disease is Treated as An Indication in 5 Approved Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
AS-1670542 DMV05SW Approved Small molecular drug [1]
Eltrombopag DMOGFIX Approved Small molecular drug [2]
GMA-161 DMGVJLB Approved (orphan drug) NA [3]
Rituximab DM1YVZT Approved Antibody [4]
SM-101 DMFX9O6 Approved (orphan drug) Small molecular drug [3]
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This Disease is Treated as An Indication in 2 Clinical Trial Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
BT595 DM2EC8A Phase 3 Protein [5]
Sc Veltuzumab DM2S8W3 Phase 1 Monoclonal antibody [6]
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This Disease is Treated as An Indication in 2 Discontinued Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
RhTPO DMCV0BZ Discontinued in Phase 3 NA [3]
BR3-Fc DMFN1WI Discontinued in Phase 2 NA [3]
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This Disease is Treated as An Indication in 1 Investigative Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
Anti-D DMG7PC0 Investigative Small molecular drug [3]
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Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)
This Disease Is Related to 39 DTT Molecule(s)
Gene Name DTT ID Evidence Level Mode of Inheritance REF
TNFSF13B TTWMIDN Limited Altered Expression [8]
CTLA4 TTI2S1D moderate Altered Expression [9]
ADAMTS13 TTUREBK Strong Biomarker [10]
ADRB3 TTMXGCW Strong Altered Expression [11]
AHCY TTE2KUJ Strong Biomarker [12]
APOH TT2OUI9 Strong Biomarker [13]
CD69 TTPQE9F Strong Altered Expression [14]
CD83 TTT9MRQ Strong Biomarker [15]
CD86 TT53XHB Strong Biomarker [15]
CXCL13 TT0NIZ1 Strong Biomarker [16]
DDX5 TTZKPVC Strong Biomarker [17]
FCER1G TTDGEC0 Strong Biomarker [18]
FCGR1A TTZK4I3 Strong Altered Expression [19]
FCGRT TTKLPHO Strong Biomarker [20]
GEM TTAZF9M Strong Genetic Variation [21]
GP1BA TTVB0Q9 Strong Altered Expression [22]
GP6 TTTJUVZ Strong Biomarker [23]
IL11 TTGUYTR Strong Biomarker [24]
IL17F TT2B6PS Strong Genetic Variation [25]
IL21 TT9QEJ6 Strong Altered Expression [26]
IL22 TTLDX4N Strong Altered Expression [27]
ITGB3 TTJA1ZO Strong Biomarker [28]
KIR2DL2 TTU0P73 Strong Biomarker [29]
KIR2DL3 TTEX3SI Strong Altered Expression [29]
KIR2DS2 TTV3CFI Strong Biomarker [29]
KIR3DL2 TTQH3N0 Strong Biomarker [21]
LTA TTP73TM Strong Genetic Variation [30]
MS4A1 TTUE541 Strong Biomarker [31]
RHD TTLCKI8 Strong Biomarker [32]
SELP TTE5VG0 Strong Biomarker [33]
STAT1 TTN7R6K Strong Genetic Variation [34]
SYK TT2HUPM Strong Biomarker [35]
TCIRG1 TTVRN05 Strong Biomarker [36]
THPO TTCG5PE Strong Biomarker [37]
TNFRSF13C TT7NJSE Strong Altered Expression [38]
TNFRSF17 TTZ3P4W Strong Altered Expression [38]
TNFSF13 TTOI1RM Strong Biomarker [39]
ICOS TTE5VP6 Definitive Biomarker [40]
TNFRSF13B TTL9OD4 Definitive Genetic Variation [41]
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⏷ Show the Full List of 39 DTT(s)
This Disease Is Related to 37 DOT Molecule(s)
Gene Name DOT ID Evidence Level Mode of Inheritance REF
ACSBG1 OTM040MW Limited Biomarker [42]
RAPH1 OTMQXW7S Limited Biomarker [42]
ACTN1 OTUCLNXH Strong Genetic Variation [43]
CD72 OTPOJID2 Strong Altered Expression [44]
CLEC1B OTO38TRG Strong Biomarker [23]
CUL9 OTPUCLZT Strong Biomarker [45]
GATA2 OTBP2QQ2 Strong Altered Expression [46]
GCHFR OTEOT8GI Strong Altered Expression [47]
GFI1B OTRDW8YO Strong Genetic Variation [48]
GP5 OT3WGPR3 Strong Biomarker [49]
GP9 OTA19OKO Strong Altered Expression [50]
HLA-DOA OTZE5Q7R Strong Genetic Variation [9]
HLA-DRB3 OT5PM9N7 Strong Genetic Variation [51]
HOXB4 OTH1HRW5 Strong Altered Expression [46]
HOXD13 OTWSC8TF Strong Biomarker [52]
IL10RA OTOX3D1D Strong Genetic Variation [53]
IL18BP OTW0LRYZ Strong Altered Expression [54]
ITGA2B OT4Y17PY Strong Altered Expression [55]
KIR2DS3 OT3PSLDL Strong Genetic Variation [29]
KIR2DS5 OTXLEN11 Strong Biomarker [29]
KIR3DL1 OTPOSXFX Strong Genetic Variation [21]
KLRD1 OTMYLOV4 Strong Biomarker [56]
KRT20 OT4RB40L Strong Biomarker [31]
MAP6 OTPUI00F Strong Genetic Variation [57]
MSC OTBRPZL5 Strong Biomarker [58]
NAP1L1 OTI7WBZV Strong Altered Expression [59]
OPTN OT2UXWH9 Strong Altered Expression [59]
PRB2 OTAD4JZ0 Strong Biomarker [60]
RNF112 OT0Q9OUQ Strong Biomarker [61]
RO60 OTLGM5A8 Strong Biomarker [62]
SCAMP1 OTFS4IKJ Strong Biomarker [63]
SCAMP2 OTFGSCHQ Strong Biomarker [63]
SLC7A4 OTAVC6QS Strong Biomarker [64]
TBXT OTHCO2F0 Strong Altered Expression [65]
TRIT1 OTCU9FS5 Strong Biomarker [66]
TSHZ1 OTYQ9ECW Strong Genetic Variation [67]
SIAH2 OTKED2XN Definitive Altered Expression [68]
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⏷ Show the Full List of 37 DOT(s)

References

1 ClinicalTrials.gov (NCT01433978) A Phase 3, Multicenter, Randomized, Double-blind, Active-controlled, Parallel-group Trial With an Open-label Extension Phase to Evaluate the Efficacy and Safety of Oral E5501 Versus Eltrombopag, in Adults With Chronic Immune Thrombocytopenia (Idiopathic Thrombocytopenic Purpura). U.S. National Institutes of Health.
2 URL: http://www.guidetopharmacology.org Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Ligand id: 6961).
3 Emerging drugs for idiopathic thrombocytopenic purpura in adults. Expert Opin Emerg Drugs. 2008 Jun;13(2):237-54.
4 Idiopathic thrombocytopenic purpura (ITP) - new era for an old disease. Rom J Intern Med. 2019 Dec 1;57(4):273-283.
5 Clinical pipeline report, company report or official report of Biotest Pharmaceuticals.
6 Subcutaneous injections of low doses of humanized anti-CD20 veltuzumab: a phase I study in chronic lymphocytic leukemia. Leuk Lymphoma. 2015 Oct 19:1-9.
7 FDA Approved Drug Products from FDA Official Website. 2009. Application Number: (NDA) 022291.
8 Effect of glucocorticoid treatment on BAFF and APRIL expression in patients with immune thrombocytopenia (ITP).Clin Immunol. 2018 Mar;188:74-80. doi: 10.1016/j.clim.2017.12.010. Epub 2017 Dec 26.
9 Association of cytotoxic T-lymphocyte antigen 4 gene with immune thrombocytopenia in Chinese Han children.Hematology. 2019 Dec;24(1):123-128. doi: 10.1080/10245332.2018.1530179. Epub 2018 Oct 14.
10 Is it quinine TTP/HUS or quinine TMA? ADAMTS13 levels and implications for therapy.J Clin Apher. 2009;24(3):115-9. doi: 10.1002/jca.20194.
11 Dysregulated megakaryocyte distribution associated with nestin(+) mesenchymal stem cells in immune thrombocytopenia.Blood Adv. 2019 May 14;3(9):1416-1428. doi: 10.1182/bloodadvances.2018026690.
12 Decreased DNA methyltransferase 3A and 3B mRNA expression in peripheral blood mononuclear cells and increased plasma SAH concentration in adult patients with idiopathic thrombocytopenic purpura.J Clin Immunol. 2008 Sep;28(5):432-9. doi: 10.1007/s10875-008-9223-2. Epub 2008 Aug 6.
13 Relevance of antiphospholipid antibody profile in the clinical outcome of ITP: a single-centre study.Hematology. 2019 Dec;24(1):134-138. doi: 10.1080/10245332.2018.1532649. Epub 2018 Oct 25.
14 Increased ADAM10 expression in patients with immune thrombocytopenia.Int Immunopharmacol. 2018 Feb;55:63-68. doi: 10.1016/j.intimp.2017.12.004. Epub 2017 Dec 22.
15 High dose dexamethasone as an alternative rescue therapy for active bleeding in children with chronic ITP: clinical and immunological effects.Platelets. 2019;30(7):886-892. doi: 10.1080/09537104.2018.1530347. Epub 2018 Oct 22.
16 Long non-coding RNA MEG3 inhibits microRNA-125a-5p expression and induces immune imbalance of Treg/Th17 in immune thrombocytopenic purpura.Biomed Pharmacother. 2016 Oct;83:905-911. doi: 10.1016/j.biopha.2016.07.057. Epub 2016 Aug 10.
17 Elucidation of the Mechanisms and Molecular Targets of Yiqi Shexue Formula for Treatment of Primary Immune Thrombocytopenia Based on Network Pharmacology.Front Pharmacol. 2019 Oct 1;10:1136. doi: 10.3389/fphar.2019.01136. eCollection 2019.
18 An (II)(b) (3) antagonist prevents thrombosis without causing Fc receptor -chain IIa-mediated thrombocytopenia.J Thromb Haemost. 2017 Nov;15(11):2230-2244. doi: 10.1111/jth.13803. Epub 2017 Oct 9.
19 Mouse immune thrombocytopenia is associated with Th1 bias and expression of activating Fc receptors.Int J Hematol. 2017 May;105(5):598-605. doi: 10.1007/s12185-016-2172-2. Epub 2016 Dec 27.
20 Phase 2 study of efgartigimod, a novel FcRn antagonist, in adult patients with primary immune thrombocytopenia.Am J Hematol. 2020 Feb;95(2):178-187. doi: 10.1002/ajh.25680. Epub 2019 Dec 10.
21 A Study of Human Killer Cell Immunoglobulin-Like Receptor and Multidrug Resistance Gene Polymorphisms in Children With Immune Thrombocytopenia.Clin Appl Thromb Hemost. 2016 Jul;22(5):429-40. doi: 10.1177/1076029615576738. Epub 2015 Mar 18.
22 Increased GPIb shedding from platelets treated with immune thrombocytopenia plasma.Int Immunopharmacol. 2019 Jan;66:91-98. doi: 10.1016/j.intimp.2018.11.011. Epub 2018 Nov 13.
23 Significant Hypo-Responsiveness to GPVI and CLEC-2 Agonists in Pre-Term and Full-Term Neonatal Platelets and following Immune Thrombocytopenia.Thromb Haemost. 2018 Jun;118(6):1009-1020. doi: 10.1055/s-0038-1646924. Epub 2018 Apr 25.
24 Platelet and peripheral white blood cell counts at diagnosis predict the response of adult immune thrombocytopenia to recombinant human interleukin-11: A retrospective, single-center, case-control study.Medicine (Baltimore). 2019 Apr;98(16):e15195. doi: 10.1097/MD.0000000000015195.
25 Assessment of IL-17F rs763780 gene polymorphism in immune thrombocytopenia.Blood Cells Mol Dis. 2019 Mar;75:20-25. doi: 10.1016/j.bcmd.2018.12.001. Epub 2018 Dec 14.
26 Clinical significance and immunobiology of IL-21 in autoimmunity.J Autoimmun. 2019 May;99:1-14. doi: 10.1016/j.jaut.2019.01.013. Epub 2019 Feb 14.
27 The potential therapeutic benefit of resveratrol on Th17/Treg imbalance in immune thrombocytopenic purpura.Int Immunopharmacol. 2019 Aug;73:181-192. doi: 10.1016/j.intimp.2019.04.061. Epub 2019 May 16.
28 Megakaryocytic dysfunction in immune thrombocytopenia is linked to autophagy.Cancer Cell Int. 2019 Mar 15;19:59. doi: 10.1186/s12935-019-0779-0. eCollection 2019.
29 The presence of KIR2DS5 confers protection against adult immune thrombocytopenia.Tissue Antigens. 2014 Mar;83(3):154-60. doi: 10.1111/tan.12295. Epub 2014 Feb 12.
30 The potential association of tumor necrosis factor-eta (252 G/A) cytokine gene polymorphism with immune thrombocytopenic purpura among Egyptian children.Hematology. 2018 Jun;23(5):299-303. doi: 10.1080/10245332.2017.1386429. Epub 2017 Oct 12.
31 Anterior ST-elevation myocardial infarction induced by rituximab infusion: A case report and review of the literature.J Clin Pharm Ther. 2017 Jun;42(3):356-362. doi: 10.1111/jcpt.12522.
32 RHD zygosity predicts degree of platelet response to anti-D immune globulin treatment in children with immune thrombocytopenia.Pediatr Blood Cancer. 2013 Sep;60(9):E106-8. doi: 10.1002/pbc.24574. Epub 2013 May 27.
33 Effect of thrombopoietin receptor agonists on markers of coagulation and P-selectin in patients with immune thrombocytopenia.Platelets. 2019;30(2):206-212. doi: 10.1080/09537104.2017.1394451. Epub 2017 Dec 7.
34 STAT1 single nucleotide polymorphisms and susceptibility to immune thrombocytopenia.Autoimmunity. 2015;48(5):305-12. doi: 10.3109/08916934.2015.1016218. Epub 2015 Feb 24.
35 Fostamatinib for the treatment of immune thrombocytopenia in adults.Am J Health Syst Pharm. 2019 May 17;76(11):789-794. doi: 10.1093/ajhp/zxz052.
36 Elevated levels of T-cell immune response cDNA 7 in patients with immune thrombocytopenia.Hematology. 2014 Dec;19(8):477-82. doi: 10.1179/1607845414Y.0000000156. Epub 2014 Mar 11.
37 Primary immune thrombocytopenia (ITP) treated with romiplostim in routine clinical practice: retrospective study from the United Kingdom ITP Registry.Eur J Haematol. 2019 May;102(5):416-423. doi: 10.1111/ejh.13221. Epub 2019 Mar 7.
38 Reduced tumour necrosis factor receptor superfamily 13C inversely correlated with tumour necrosis factor superfamily 13B in patients with immune thrombocytopenia.Br J Haematol. 2014 Sep;166(5):783-91. doi: 10.1111/bjh.12958. Epub 2014 May 30.
39 B-cell-activating factor, a proliferation inducing ligand and co-stimulatory molecules in the pathogenesis of immune thrombocytopenia in childhood.Blood Coagul Fibrinolysis. 2016 Jul;27(5):494-9. doi: 10.1097/MBC.0000000000000144.
40 Adult Primary Immune Thrombocytopenia: Spleen Histology Findings and Outcomes According to Rituximab Use Based on Analysis of 41 Cases.Am J Surg Pathol. 2018 Mar;42(3):401-412. doi: 10.1097/PAS.0000000000000994.
41 A gain-of-function mutation in TNFRSF13B is a candidate for predisposition to familial or sporadic immune thrombocytopenia.J Thromb Haemost. 2017 Nov;15(11):2259-2269. doi: 10.1111/jth.13806. Epub 2017 Sep 13.
42 Use of eltrombopag for patients 65years old or older with immune thrombocytopenia.Eur J Haematol. 2020 Mar;104(3):259-270. doi: 10.1111/ejh.13370. Epub 2020 Feb 3.
43 Familial macrothrombocytopenia due to a double mutation in cis in the alpha-actinin 1 gene (ACTN1), previously considered to be chronic immune thrombocytopenic purpura.Pediatr Blood Cancer. 2018 Dec;65(12):e27418. doi: 10.1002/pbc.27418. Epub 2018 Aug 19.
44 Upregulation of CD72 expression on CD19(+) CD27(+) memory Bcells by CD40L in primary immune thrombocytopenia.Br J Haematol. 2017 Jul;178(2):308-318. doi: 10.1111/bjh.14671. Epub 2017 Apr 17.
45 Idiopathic thrombocytopenic purpura (ITP): is there a genetic predisposition?.Pediatr Blood Cancer. 2006 Oct 15;47(5 Suppl):678-80. doi: 10.1002/pbc.21005.
46 Gene expression profiling identifies HOXB4 as a direct downstream target of GATA-2 in human CD34+ hematopoietic cells.PLoS One. 2012;7(9):e40959. doi: 10.1371/journal.pone.0040959. Epub 2012 Sep 24.
47 Decreased IL-35 levels in patients with immune thrombocytopenia.Hum Immunol. 2014 Aug;75(8):909-13. doi: 10.1016/j.humimm.2014.06.019. Epub 2014 Jun 30.
48 Macrothrombocytopenia associated with a rare GFI1B missense variant confounding the presentation of immune thrombocytopenia.Pediatr Blood Cancer. 2019 Sep;66(9):e27874. doi: 10.1002/pbc.27874. Epub 2019 Jun 17.
49 Glycoprotein V is a relevant immune target in patients with immune thrombocytopenia.Haematologica. 2019 Jun;104(6):1237-1243. doi: 10.3324/haematol.2018.211086. Epub 2019 Mar 28.
50 A novel mutation in the GP1BA gene in Bernard-Soulier syndrome.Blood Coagul Fibrinolysis. 2020 Jan;31(1):83-86. doi: 10.1097/MBC.0000000000000868.
51 Pregnancy-associated autoimmune neonatal thrombocytopenia: role of maternal HLA genotype.Br J Haematol. 1999 Mar;104(4):878-85. doi: 10.1046/j.1365-2141.1999.01270.x.
52 PD-1/PD-L1 pathway activation restores the imbalance of Th1/Th2 and treg/Th17 cells subtypes in immune thrombocytopenic purpura patients.Medicine (Baltimore). 2019 Oct;98(43):e17608. doi: 10.1097/MD.0000000000017608.
53 Novel exonic mutation inducing aberrant splicing in the IL10RA gene and resulting in infantile-onset inflammatory bowel disease: a case report.BMC Gastroenterol. 2016 Jan 28;16:10. doi: 10.1186/s12876-016-0424-5.
54 Detection of expression of IL-18 and its binding protein in Egyptian pediatric immune thrombocytopenic purpura.Platelets. 2014;25(3):193-6. doi: 10.3109/09537104.2013.784734. Epub 2013 Apr 4.
55 Antigenic Mimicry in Paraneoplastic Immune Thrombocytopenia.Front Immunol. 2019 Mar 22;10:523. doi: 10.3389/fimmu.2019.00523. eCollection 2019.
56 NK cell compartment in the peripheral blood and spleen in adult patients with primary immune thrombocytopenia.Clin Immunol. 2017 Apr;177:18-28. doi: 10.1016/j.clim.2015.11.005. Epub 2015 Nov 18.
57 FCGR2C genotyping by pyrosequencing reveals linkage disequilibrium with FCGR3A V158F and FCGR2A H131R polymorphisms in a Caucasian population.MAbs. 2012 Nov-Dec;4(6):784-7. doi: 10.4161/mabs.22287. Epub 2012 Oct 2.
58 CB2 Receptor Stimulation and Dexamethasone Restore the Anti-Inflammatory and Immune-Regulatory Properties of Mesenchymal Stromal Cells of Children with Immune Thrombocytopenia.Int J Mol Sci. 2019 Feb 28;20(5):1049. doi: 10.3390/ijms20051049.
59 Thalidomide induce response in patients with corticosteroid-resistant or relapsed ITP by upregulating Neuropilin-1 expression.Int Immunopharmacol. 2019 Jul;72:437-444. doi: 10.1016/j.intimp.2019.04.041.
60 Akt-mediated platelet apoptosis and its therapeutic implications in immune thrombocytopenia.Proc Natl Acad Sci U S A. 2018 Nov 6;115(45):E10682-E10691. doi: 10.1073/pnas.1808217115. Epub 2018 Oct 18.
61 Biologic false-positive serologic tests for syphilis and other serologic abnormalities in autoimmune hemolytic anemia and thrombocytopenic purpura.Medicine (Baltimore). 1989 Mar;68(2):67-84. doi: 10.1097/00005792-198903000-00001.
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64 Genetic analysis of autoantibodies in idiopathic thrombocytopenic purpura reveals evidence of clonal expansion and somatic mutation.Blood. 2002 Aug 15;100(4):1388-98.
65 Aberrant expression of RUNX3 in patients with immune thrombocytopenia.Int Immunopharmacol. 2015 Sep;28(1):252-6. doi: 10.1016/j.intimp.2015.06.008. Epub 2015 Jun 17.
66 Profiling of miRNA expression in immune thrombocytopenia patients before and after Qishunbaolier (QSBLE) treatment.Biomed Pharmacother. 2015 Oct;75:196-204. doi: 10.1016/j.biopha.2015.07.022. Epub 2015 Aug 18.
67 CNR2 functional variant (Q63R) influences childhood immune thrombocytopenic purpura.Haematologica. 2011 Dec;96(12):1883-5. doi: 10.3324/haematol.2011.045732. Epub 2011 Aug 9.
68 The expression and function of E3 ligase SIAH2 in acute T lymphoblastic leukemia.Leuk Res. 2016 Mar;42:28-36. doi: 10.1016/j.leukres.2016.01.013. Epub 2016 Jan 28.