Details of Disease

General Information of Disease (ID: DISLZ0VW)

Disease Name	Thrombotic microangiopathy
Disease Class	3B65: Thrombotic microangiopathy
Definition	The syndromes of microangiopathic hemolytic anemia, thrombocytopenia, and variable signs of organ impairment, due to platelet aggregation in the microcirculation.
Disease Hierarchy	DIS9X3H6: Coagulation defect DISLZ0VW: Thrombotic microangiopathy
ICD Code	ICD-11 ICD-11: 3B65 ICD-10 ICD-10: M31.1
Disease Identifiers	MONDO ID MONDO_0019737 MESH ID D057049 UMLS CUI C2717961 MedGen ID 403479 Orphanet ID 93573 SNOMED CT ID 126729006

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)

This Disease is Treated as An Indication in 3 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Narsoplimab	DM5KD99	Phase 3	Monoclonal antibody	[1]
Nomacopan	DML6E6G	Phase 3	Recombinant protein	[2]
OMS721	DMK6QBZ	Phase 3	NA	[3]
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Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

This Disease Is Related to 9 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
VEGFA	TT3LJ9K	Limited	Biomarker	[4]
VWF	TT3SZBT	Limited	Genetic Variation	[5]
C5	TTKANGO	Disputed	Biomarker	[6]
THBD	TTAPV67	Disputed	Biomarker	[7]
C3	TTJGY7A	Strong	Biomarker	[8]
CFI	TT6ATLX	Strong	Genetic Variation	[9]
FGG	TTR31L7	Strong	Biomarker	[10]
PDGFC	TTOABM9	Strong	Biomarker	[11]
GP6	TTTJUVZ	Definitive	Altered Expression	[12]
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⏷ Show the Full List of 9 DTT(s)

This Disease Is Related to 1 DTP Molecule(s)

Gene Name	DTP ID	Evidence Level	Mode of Inheritance	REF
SLC25A10	DTJYKDQ	Definitive	Altered Expression	[12]
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This Disease Is Related to 7 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
SH3BP4	OTVIRKW7	moderate	Biomarker	[13]
CABIN1	OT4G5CIK	Strong	Genetic Variation	[14]
CFHR1	OT72R16T	Strong	Genetic Variation	[15]
CFHR3	OTYL8SDO	Strong	Genetic Variation	[15]
PIGA	OT51UWUR	Strong	Biomarker	[16]
CLEC1B	OTO38TRG	Definitive	Altered Expression	[12]
DGKE	OTWS86AS	Definitive	Genetic Variation	[17]
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⏷ Show the Full List of 7 DOT(s)

References

1	ClinicalTrials.gov (NCT02222545) A Phase 2, Uncontrolled, Three-Stage, Dose-Escalation Cohort Study to Evaluate the Safety, Pharmacokinetics, Pharmacodynamics, Immunogenicity, and Clinical Activity of OMS721 in Adults With Thrombotic Microangiopathies. U.S.National Institutes of Health.
2	ClinicalTrials.gov (NCT04784455) Multicentre Study of Nomacopan (rVA576) in Paediatric Haematopoietic Stem-Cell Transplant Associated Thrombotic Microangiopathy. U.S.National Institutes of Health.
3	Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
4	Renal thrombotic microangiopathy during nintedanib treatment for idiopathic pulmonary fibrosis?"Inoue D. Honda K
5	Caplacizumab for relapsing thrombotic thrombocytopenic purpura.Pediatr Nephrol. 2019 Sep;34(9):1625-1628. doi: 10.1007/s00467-019-04281-z. Epub 2019 Jun 8.
6	Complement C5-inhibiting therapy for the thrombotic microangiopathies: accumulating evidence, but not a panacea.Clin Kidney J. 2017 Oct;10(5):600-624. doi: 10.1093/ckj/sfx081. Epub 2017 May 8.
7	The fifth epidermal growth factor-like region of thrombomodulin exerts cytoprotective function and prevents SOS in a murine model.Bone Marrow Transplant. 2017 Jan;52(1):73-79. doi: 10.1038/bmt.2016.195. Epub 2016 Jul 18.
8	Murine systemic thrombophilia and hemolytic uremic syndrome from a factor H point mutation.Blood. 2017 Mar 2;129(9):1184-1196. doi: 10.1182/blood-2016-07-728253. Epub 2017 Jan 5.
9	C3 glomerulonephritis and thrombotic microangiopathy of renal allograft after pulmonary infection in a male with concomitant two complement factor I gene variations: a case report.BMC Nephrol. 2018 Jun 25;19(1):148. doi: 10.1186/s12882-018-0952-z.
10	Plasma fibrinogen gamma' chain content in the thrombotic microangiopathy syndrome.J Thromb Haemost. 2007 Jan;5(1):62-9. doi: 10.1111/j.1538-7836.2006.02270.x. Epub 2006 Oct 13.
11	PDGF-C mediates glomerular capillary repair.Am J Pathol. 2010 Jul;177(1):58-69. doi: 10.2353/ajpath.2010.091008. Epub 2010 May 20.
12	Elevated plasma levels of soluble C-type lectin-like receptor 2 (CLEC2) in patients with thrombotic microangiopathy.Thromb Res. 2019 Jun;178:54-58. doi: 10.1016/j.thromres.2019.03.018. Epub 2019 Mar 28.
13	Thrombocytopenia-Associated Multiple Organ Failure and Acute Kidney Injury.Crit Care Clin. 2015 Oct;31(4):661-74. doi: 10.1016/j.ccc.2015.06.004. Epub 2015 Aug 7.
14	Antiphospholipid Syndrome Nephropathy and Other Thrombotic Microangiopathies Among Patients With Systemic Lupus Erythematosus.Adv Chronic Kidney Dis. 2019 Sep;26(5):376-386. doi: 10.1053/j.ackd.2019.08.012.
15	A Heterozygous CFHR3-CFHR1 Gene Deletion in a Pediatric Patient With Transplant-associated Thrombotic Microangiopathy Who was Treated With Eculizumab.J Pediatr Hematol Oncol. 2018 Nov;40(8):e544-e546. doi: 10.1097/MPH.0000000000000986.
16	Modified Ham test for atypical hemolytic uremic syndrome.Blood. 2015 Jun 4;125(23):3637-46. doi: 10.1182/blood-2015-02-629683. Epub 2015 Apr 10.
17	Diacylglycerol kinase epsilon nephropathy: late diagnosis and therapeutic implications.Clin Kidney J. 2019 May 14;12(5):641-644. doi: 10.1093/ckj/sfz043. eCollection 2019 Oct.