Atlas One · Host Biology · Scale 07 — System

Paroxysmal Nocturnal Hemoglobinuria

PNH is a clonal PIGA disorder of GPI-anchor synthesis; GPI-deficient RBCs lack CD55/CD59 → complement MAC lysis + C5a-driven thrombosis; eculizumab and ravulizumab (anti-C5 mAbs) normalize hemolysis and reduce thrombosis 90%; iptacopan (oral factor B inhibitor) also approved.

Also known as: PNH, paroxysmal nocturnal hemoglobinuria, PIGA mutation, GPI-anchor deficiency, CD55/CD59 deficiency, Marchiafava-Micheli disease

Scale 07 — SystemScale
draftStatus
2026-06-07Last Reviewed
This is an auto-generated stub page built from atlas entry frontmatter. A richer hand-crafted page is planned. See the atlas source for the full entry including detailed body sections.
pnh

Paroxysmal Nocturnal Hemoglobinuria

PNH is a clonal PIGA disorder of GPI-anchor synthesis; GPI-deficient RBCs lack CD55/CD59 → complement MAC lysis + C5a-driven thrombosis; eculizumab and ravulizumab (anti-C5 mAbs) normalize hemolysis and reduce thrombosis 90%; iptacopan (oral factor B inhibitor) also approved.

Entry Metadata

FieldValue
IDpnh
NameParoxysmal Nocturnal Hemoglobinuria
Statusdraft
Last reviewed2026-06-07
Atlas01-human
Scale07-system

Cross-Atlas Connections

connects-toComplement C5 connects-toBone Marrow connects-toAplastic Anemia connects-toC5Ar1 connects-toFactor H connects-toErythrocyte connects-toVenous Thromboembolism connects-toLiver connects-toHeparin Induced Thrombocytopenia connects-toMds connects-toPlatelet connects-toAhus connects-toInherited Thrombophilia connects-toMacrophage connects-toComplement C3 connects-toAml connects-toDisseminated Intravascular Coagulation connects-toNitric Oxide connects-toKidney connects-toImmune System connects-toHemoglobin connects-toNeutrophil connects-toPulmonary Arterial Hypertension connects-toSmooth Muscle Cell connects-toIron connects-toT Cytotoxic Cell connects-toPotassium connects-toThrombin connects-toNatural Killer Cell connects-toSpleen connects-toHydrogen connects-toEndothelial Cell connects-toPhoton connects-toBrain connects-toVon Willebrand Factor connects-toElectron connects-toLarge Intestine connects-toHeart connects-toNeuron connects-toReproductive System connects-toStomach connects-toAntibody connects-toHepatocyte connects-toSkin connects-toCkd connects-toIron Deficiency Anemia connects-toErythropoietin connects-toStroke connects-toHepcidin connects-toRegulatory T Cell connects-toNf Kb connects-toSepsis connects-toHcc connects-toHeart Failure connects-toAnemia Of Chronic Disease connects-toMajor Depressive Disorder connects-toNeisseria Meningitidis connects-toStreptococcus Pneumoniae connects-toGvhd connects-toDigestive System connects-toNervous System connects-toGeneralized Anxiety Disorder connects-toIntegumentary System connects-toRespiratory System connects-toCardiovascular System connects-toMusculoskeletal System connects-toRenal System connects-toNeisseria Meningitidis connects-toTargeted Therapy connects-toLymphatic System connects-toWarfarin connects-toCancer Chemotherapy connects-toSickle Cell Disease connects-toHepatic Lobule connects-toGlomerulus connects-toArterial Wall connects-toThrombotic Thrombocytopenic Purpura connects-toMyasthenia Gravis connects-toNmo connects-toPlacenta connects-toAntiphospholipid Syndrome connects-toEssential Thrombocythemia connects-toCovid 19 Disease connects-toFibrinogen connects-toMyelofibrosis connects-toPolycythemia Vera connects-toPf4 connects-toFerroportin connects-toTlr4 connects-toIfn Gamma connects-toPerforin connects-toProtein C connects-toThrombopoietin connects-toS100A8 A9 connects-toRage connects-toTnf Alpha connects-toNlrp3 Inflammasome connects-toHif 1Alpha connects-toEndothelin 1 connects-toAngiopoietin connects-toRunx1 connects-toJak1 2 connects-toAkt connects-toErk1 2 connects-toGalectin 3 connects-toCgas Sting connects-toStat3 connects-toFoxo connects-toStat1 connects-toSmad4 connects-toGsk 3B connects-toMtor connects-toPik3Ca connects-toSrc Kinase connects-toAmpk connects-toAutophagy connects-toCcr5 connects-toDnmt3A connects-toCxcl12 connects-toIl 1B connects-toIl 33 connects-toIl 17A connects-toMhc Class Ii connects-toIl 2 connects-toCalcium connects-toMu Opioid Receptor connects-toXanthine Oxidase connects-toIl 6 connects-toAdamts13 connects-toTroponin Complex connects-toIl 10 connects-toProstaglandins connects-toIl 4 connects-toZinc connects-toIl 13 connects-toType I Interferon connects-toPotassium connects-toLeptin connects-toAdiponectin connects-toResistin connects-toIl 12 connects-toIl 5 connects-toIge connects-toIl 23 connects-toDendritic Cell connects-toB Cell connects-toT Helper Cell connects-toPlasma Cell connects-toTransferrin connects-toC1 Esterase Inhibitor connects-toOsteopontin connects-toMast Cell connects-toTslp connects-toBradykinin connects-toHistamine connects-toPeriostin connects-toMelatonin connects-toProlactin connects-toTestosterone connects-toSerotonin connects-toOxytocin connects-toVasopressin connects-toSelenium connects-toIodine connects-toSodium connects-toMagnesium connects-toCopper connects-toPhosphorus connects-toChloride connects-toCarbon connects-toNitrogen connects-toOxygen connects-toSulfur connects-toPd 1 connects-toGlp 1 connects-toAngiotensin Ii connects-toWnt Beta Catenin connects-toRankl connects-toVegf connects-toFibronectin connects-toNotch connects-toIgf 1 connects-toActivin A connects-toTgf Beta connects-toCgrp connects-toCalcitonin connects-toSubstance P connects-toInsulin Receptor connects-toAldosterone connects-toAndrogen Receptor connects-toNorepinephrine connects-toAdrenomedullin connects-toBdnf

Sources