Disseminated intravascular coagulation (DIC)
STEP 3 deepened 2026-05-16; citations re-verified 2026-05-22: §5.5.1 effect sizes wired (ISTH overt-DIC score >=5 threshold + mortality correlation Taylor/Wada; SCARLET rTM 28-day mortality 26.8% vs 29.4% NS PMID 31104069; APL early DIC-hemorrhagic death ~5-10% despite CR >90% ELN APL PMID 30803991; sepsis-DIC ~2x mortality Levi PMID 10451465). §5.5.2 Factor-VIII pivot (DIC vs liver), TTP/HIT/VKDC/dilutional/acquired-haemophilia pivots, cross-routing (heme.ttp, workup.acute_leukemia, sepsis bundle). 2026-05-22 PMID remediation: replaced mis-attributed Wada 23889640->23379279, Levi 24847960->10451465 (NEJM 1999 DIC review), SCARLET 31573641->31104069, Sanz/APL 19740935->30803991 (ELN APL 2019); last_reconciled 2026-05-22. NEW dossier — manifest repointed. NEXT STEPS: (1) author manifest at prisma/seed/manifests/heme.dic.v1.ts; (2) RxCUI validation for heparin (UFH), enoxaparin, vitamin K, PCC, tranexamic acid, ATRA, arsenic trioxide (blood products lack reliable RxCUIs); (3) calculator gaps — ISTH DIC score may be in panel.coag; verify exposure. Treat-cause-first principle explicit; phenotype-directed therapy (bleeding vs thrombotic vs APL) captured. Sibling differentiation from heme.ttp.core.v1 covers 5 features + PLASMIC routing.
Entry points (5)
- lab_abnormalityThrombocytopenia + abnormal PT/aPTT in critically ill patient (ISTH 2009)thrombocytopenia_with_coag_abnormality
- symptomBleeding from ≥2 sites (line, mucosal, surgical, wound) (ISTH 2009)bleeding_from_multiple_sites
- symptomDigital ischemia, purpura fulminans, organ dysfunction with consumptive coagulopathy (ISTH 2009)microthrombosis_digital_ischemia_or_purpura_fulminans
- historyAML M3 / APL with bleeding diathesis (ELN APL 2019)apl_with_bleeding
- historyObstetric emergency (HELLP, AFE, abruption, retained fetus) with coagulopathy (ISTH 2009)obstetric_emergency_dic
Required inputs (16)
- agerequireddemographic • used at CONTEXTRisk profile + cause vary by age (ISTH 2009)
- sex_pregnancy_statusrequireddemographic • used at CONTEXTObstetric DIC pathway different (ISTH 2009)
- sbprequiredvital • used at CONTEXTSeptic shock common precipitant (SSC 2021)
- temperaturerequiredvital • used at CONTEXTSeptic precipitant (SSC 2021)
- cbc_with_smearrequiredlab • used at INITIAL_WORKUPPlatelets + schistocytes (TTP/HUS DDx) (ISTH 2009)
- pt_inr_apttrequiredlab • used at INITIAL_WORKUPISTH score; coagulopathy magnitude (ISTH 2009)
- fibrinogenrequiredlab • used at INITIAL_WORKUPISTH score component; replacement target (ISTH 2009; BSH 2020)
- d_dimer_or_fdprequiredlab • used at INITIAL_WORKUPISTH score component (ISTH 2009)
- lactaterequiredlab • used at INITIAL_WORKUPSepsis severity (SSC 2021)
- lft_creatininerequiredlab • used at INITIAL_WORKUPLiver failure DDx; renal involvement (ISTH 2009)
- adamts13lab • used at BRANCHING_WORKUPExclude TTP (ISTH 2024)
- hit_4ts_pf4_assaylab • used at BRANCHING_WORKUPExclude HIT (ISTH 2024)
- blood_culturesrequiredlab • used at INITIAL_WORKUPSource identification (SSC 2021)
- precipitant_knownrequiredhistory • used at CONTEXTSepsis, trauma, malignancy, obstetric, pancreatitis, transfusion, snake bite, hepatic (ISTH 2009)
- current_medsrequiredmedication • used at CONTEXTAnticoag baseline; chemo (APL on ATRA) (ISTH 2009; ELN APL 2019)
- imaging_for_sourceimaging • used at BRANCHING_WORKUPCT for abscess / hematoma / placental (ISTH 2009)
12-phase flow (12)
- 1FRAMEConfirm DIC via ISTH 2001 overt-DIC score — Bayesian threshold ≥5 = overt DIC (validated, high sensitivity/specificity vs reference standard; each point correlates with rising ICU mortality — Taylor ISTH 2001 PMID 11816725, Wada DIC harmonization PMID 23379279); <5 = non-overt → serial re-scoring (DIC is dynamic). Rule out look-alikes (liver, ITP, TTP, HIT, dilutional)inputs: cbc_with_smear, pt_inr_aptt, fibrinogen, d_dimer_or_fdpadvance: ISTH score calculated; differential narrowed
- 2ENTRYAbnormal coags + thrombocytopenia in critically ill, bleeding multiple sites, microthrombi, purpura fulminans, suspected APL, OB emergencyinputs: ageadvance: Entry trigger captured
- 3CONTEXTPrecipitant, baseline coags, transfusion, pregnancy, malignancyinputs: precipitant_known, sex_pregnancy_status, current_medsadvance: Context complete
- 4RED_FLAGSLife-threatening bleeding, purpura fulminans (esp meningococcemia), APL with bleeding, ICH, OB emergencyinputs: sbpactions: workup.bacterial_meningitis, workup.acute_leukemiaadvance: Acute red flags addressed
- 5INITIAL_WORKUPCBC + smear, PT/INR/aPTT, fibrinogen, D-dimer/FDP, LFT, CMP, lactate, source cultures + imaginginputs: cbc_with_smear, pt_inr_aptt, fibrinogen, d_dimer_or_fdp, lft_creatinine, lactate, blood_culturesactions: panel.cbc, panel.coag, panel.lft, panel.renal, workup.dicadvance: Stage-1 returned + ISTH score
- 6BRANCHING_WORKUPSchistocyte review for TTP/HUS, ADAMTS13, HIT 4Ts + PF4 assay, fibrinogen trends, source workup (CT for abscess, OB ultrasound)inputs: adamts13, hit_4ts_pf4_assay, imaging_for_sourceactions: workup.endocarditis, workup.ttp_standalone, 4tsadvance: Differential refined
- 7DIFFERENTIALNamed Bayesian pivots — Factor VIII is the load-bearing discriminator: normal/HIGH in DIC (acute-phase) vs LOW in hepatic synthetic failure when PT/fibrinogen abnormal in both. TTP: schistocytes ++, ADAMTS13 <10%, NORMAL PT/fibrinogen (DIC has abnormal coags) → route heme.ttp.core.v1. HIT: heparin day 5-10 + thrombosis + 4Ts, normal fibrinogen. Vitamin-K deficiency: isolated PT, corrects with vitamin K. Dilutional: massive-transfusion history. Acquired haemophilia: isolated aPTT + abnormal mixing study — Levi NEJM 1999 (PMID 10451465); Wada DIC harmonization (PMID 23379279)advance: Diagnosis confirmed
- 8RISK_STRATIFICATIONISTH overt vs non-overt; phenotype (bleeding / thrombotic / mixed); SOFA if ICUinputs: cbc_with_smear, pt_inr_aptt, fibrinogen, d_dimer_or_fdpactions: calc.sofa, calc.qsofaadvance: Phenotype + severity tier documented
- 9TREATMENTTREAT UNDERLYING CAUSE first (sepsis source control + abx; surgical / OB delivery; ATRA + ATO for APL); supportive replacement for active bleeding (FFP 10-15 mL/kg, cryoprecipitate 1 unit/10 kg target fibrinogen >150, platelets target ≥50 or ≥100 in CNS bleed, PCC for VKDC); heparin in chronic/thrombotic phenotype; AVOID TXA unless proven hyperfibrinolysis (APL/obstetric — otherwise catastrophic thrombosis risk); recombinant thrombomodulin did NOT significantly reduce 28-day mortality in sepsis-associated coagulopathy (~26.8% vs 29.4%, SCARLET JAMA 2019 PMID 31104069) — region-specific use only; APL: start ATRA at first suspicion (before genetic confirmation) — early DIC-driven hemorrhagic death is the leading induction-mortality cause (~5-10%) despite CR >90% with ATRA+ATO (ELN APL 2019 PMID 30803991); sepsis-DIC ~2x mortality vs sepsis alone (Levi NEJM 1999 PMID 10451465)inputs: precipitant_known, fibrinogenadvance: Treat-cause + phenotype-directed plan documented
- 10DISPOSITIONICU for severe bleeding / hemodynamic instability / source needing urgent interventionadvance: Disposition documented
- 11MONITORINGCoags + fibrinogen + platelets q6-12 h, ISTH score serial, source responseadvance: Monitoring orders documented
- 12FOLLOWUPHematology, oncology, OB, ID per cause; thromboprophylaxis once safeadvance: Follow-up scheduled