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hemophilia-a
Hemophilia A
Hemophilia A is X-linked FVIII deficiency (F8 gene; Xq28); severe <1 IU/dL → joint/muscle hemorrhage. Emicizumab (bispecific FIXa/FX mAb; HAVEN-3: ABR 0.3 vs 22.9; FDA 2017) replaced prophylactic FVIII as standard of care in inhibitor and non-inhibitor severe HA.
Entry Metadata
| Field | Value |
|---|---|
| ID | hemophilia-a |
| Name | Hemophilia A |
| Status | draft |
| Last reviewed | 2026-06-07 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
connects-toVon Willebrand Factor
connects-toImmunoglobulin G
connects-toThrombin
connects-toProtein C
connects-toVenous Thromboembolism
connects-toHepatocyte
connects-toAntithrombin
connects-toInherited Thrombophilia
connects-toMusculoskeletal System
connects-toEndothelial Cell
connects-toPlatelet
connects-toDisseminated Intravascular Coagulation
connects-toImmune Thrombocytopenia
connects-toThrombotic Thrombocytopenic Purpura
connects-toLiver
connects-toStroke
connects-toAntiphospholipid Syndrome
connects-toFibrinogen
connects-toBrain
connects-toHepatitis C
connects-toReproductive System
connects-toImmune System
connects-toErythrocyte
connects-toCalcium
connects-toT Helper Cell
connects-toMacrophage
connects-toCollagen
connects-toRegulatory T Cell
connects-toB Cell
connects-toIron
connects-toKidney
connects-toLarge Intestine
connects-toPhoton
connects-toSkin
connects-toBone Marrow
connects-toHiv Aids
connects-toElectron
connects-toEye
connects-toNeuron
connects-toOsteoblast
connects-toSmooth Muscle Cell
connects-toAntibody
connects-toPeripheral Nerve
connects-toHepatitis B
connects-toOsteoclast
connects-toOsteoporosis
connects-toDendritic Cell
connects-toFibroblast
connects-toT Cytotoxic Cell
connects-toIron Deficiency Anemia
connects-toNf Kb
connects-toAnemia Of Chronic Disease
connects-toSepsis
connects-toOpioid Use Disorder
connects-toMajor Depressive Disorder
connects-toHeart Failure
connects-toNeuropathic Pain
connects-toCkd
connects-toHypertension
connects-toWound Healing
connects-toDigestive System
connects-toGeneralized Anxiety Disorder
connects-toNervous System
connects-toRenal System
connects-toIntegumentary System
connects-toRespiratory System
connects-toCardiovascular System
connects-toIbuprofen
connects-toTargeted Therapy
connects-toLymphatic System
connects-toPrion Protein
connects-toCortical Bone
connects-toHepatic Lobule
connects-toSickle Cell Disease
connects-toHeparin Induced Thrombocytopenia
connects-toRheumatoid Arthritis
connects-toDlbcl
connects-toHcc
connects-toGerminal Center
connects-toArterial Wall
connects-toBreast Cancer
connects-toGlomerulus
connects-toCovid 19 Disease
connects-toRankl
connects-toTnf Alpha
connects-toVegf
connects-toIl 1B
connects-toPdgf
connects-toTgf Beta
connects-toCcl2
connects-toHif 1Alpha
connects-toTlr4
connects-toAngiopoietin
connects-toCaspase 3
connects-toIl 6
connects-toIl 10
connects-toNlrp3 Inflammasome
connects-toFerroportin
connects-toS100A8 A9
connects-toFcrn
connects-toCtla 4
connects-toMyd88
connects-toJak1 2
connects-toErk1 2
connects-toGalectin 3
connects-toSmad4
connects-toStat3
connects-toFoxo
connects-toStat1
connects-toCgas Sting
connects-toAkt
connects-toGsk 3B
connects-toPerforin
connects-toPik3Ca
connects-toMtor
connects-toSrc Kinase
connects-toCcr5
connects-toDnmt3A
connects-toCxcl12
connects-toComplement C3
connects-toIl 33
connects-toCalcineurin
connects-toType I Interferon
connects-toHemoglobin
connects-toMu Opioid Receptor
connects-toMhc Class Ii
connects-toIl 2
connects-toIl 4
connects-toProstaglandins
connects-toXanthine Oxidase
connects-toIl 13
connects-toCalcium
connects-toIl 17A
connects-toNitric Oxide
connects-toPd 1
connects-toRegulatory T Cell
connects-toBaff
connects-toHepcidin
connects-toLeptin
connects-toAdiponectin
connects-toResistin
connects-toIfn Gamma
connects-toIl 12
connects-toIl 5
connects-toIl 23
connects-toIge
connects-toPlasma Cell
connects-toMast Cell
connects-toComplement C5
connects-toC5Ar1
connects-toFactor H
connects-toTransferrin
connects-toTslp
connects-toBradykinin
connects-toErythropoietin
connects-toC1 Esterase Inhibitor
connects-toHistamine
connects-toPeriostin
connects-toMelatonin
connects-toTestosterone
connects-toSerotonin
connects-toOxytocin
connects-toVasopressin
connects-toProlactin
connects-toZinc
connects-toSelenium
connects-toIodine
connects-toSodium
connects-toMagnesium
connects-toChloride
connects-toNitrogen
connects-toPhosphorus
connects-toCarbon
connects-toCopper
connects-toHydrogen
connects-toGlp 1
connects-toAngiotensin Ii
connects-toWnt Beta Catenin
connects-toFibronectin
connects-toNotch
connects-toIgf 1
connects-toActivin A
connects-toCgrp
connects-toCalcitonin
connects-toSubstance P
connects-toInsulin Receptor
connects-toAldosterone
connects-toAndrogen Receptor
connects-toNorepinephrine
connects-toAdrenomedullin
connects-toBdnf
connects-toOsteopontin
connects-toFgfr
Sources
- Oldenburg J, Mahlangu JN, Kim B, et al. Emicizumab prophylaxis in hemophilia A with inhibitors. N Engl J Med. 2017;377(9):809-818. · PubMed 28691557
- Mahlangu J, Oldenburg J, Paz-Priel I, et al. Emicizumab prophylaxis in patients who have hemophilia A without inhibitors. N Engl J Med. 2018;379(9):811-822. · PubMed 30157389
- Pipe SW, Leebeek FW, Recht M, et al. Once-monthly subcutaneous fitusiran versus on-demand bypassing agent for haemophilia A or B with inhibitors (ATLAS-INH): a multicentre, open-label, randomised phase 3 trial. Lancet. 2023;401(10386):1427-1439. · PubMed 37003297