All dossiers
heme.itp.core.v1
Immune Thrombocytopenia (ITP) — initial diagnosis & acute Rx
hematologyacutesubacuteadultpediatricacuteinpatientoutpatient
Manifest is full but no _design-brief and no problem package on disk. Per heuristic still INTEGRATED via manifest + atoms + evidence + linked workup + ≥1 calculator (4Ts). RxCUIs absent from manifest; pending validation via scripts/research/rxnav-validate.ts. Chronic transition routes to heme.itp.chronic.v1 (currently SCAFFOLDED).
Entry points (3)
- lab_abnormalityIsolated thrombocytopenia (platelet count <100k) with otherwise normal CBC — ASH 2019 Neunertisolated_thrombocytopenia
- symptomPetechiae / purpura / mucocutaneous bleeding — ASH 2019 Neunertmucocutaneous_bleeding
- symptomMajor bleeding (intracranial/GI) — emergency presentation — ASH 2019 Neunert; BSH 2024major_bleeding
Required inputs (9)
- agerequireddemographic • used at CONTEXTPediatric ITP often self-limited (70–80% remit ≤6mo); adult management distinct — ASH 2019 Neunert
- platelet_countrequiredlab • used at ENTRYSeverity tier: <30k high-risk; <10k major bleed risk — ASH 2019 Neunert
- cbc_with_diffrequiredlab • used at INITIAL_WORKUPRule out other cytopenias (Evans, MDS, leukemia, aplastic) — ASH 2019 Neunert; BSH 2024
- peripheral_smearrequiredlab • used at INITIAL_WORKUPRule out pseudothrombocytopenia, schistocytes (TTP/HUS), blasts — ASH 2019 Neunert; Provan 2019
- coag_panelrequiredlab • used at INITIAL_WORKUPRule out DIC; baseline before procedures — ASH 2019 Neunert
- recent_drugsrequiredhistory • used at CONTEXTDrug-induced ITP (heparin → 4Ts; quinine, vanc, sulfa, etc.) — ASH 2019 Neunert; Provan 2019
- autoimmunehistory • used at CONTEXTSLE, APS, CVID screen → secondary ITP — ASH 2019 Neunert; BSH 2024
- recent_infectionhistory • used at CONTEXTViral (HIV, HCV, H. pylori, EBV, CMV); post-vaccination — ASH 2019 Neunert
- pregnancyhistory • used at CONTEXTPregnancy-associated ITP vs gestational thrombocytopenia — ASH 2019 Neunert; NICE 2024
12-phase flow (12)
- 1FRAMEConfirm isolated thrombocytopenia + exclude pseudothrombocytopenia (citrate tube), drug-induced, secondary causes — ASH 2019 Neunert; Provan 2019inputs: platelet_count, cbc_with_diff, peripheral_smearadvance: Primary ITP diagnosis remains after exclusions
- 2ENTRYStratify by platelet count + bleeding (ITP-BAT) — ASH 2019 Neunert; Provan 2019inputs: platelet_countadvance: Severity + bleeding tier set
- 3CONTEXTCapture drug history (heparin → 4Ts), autoimmune, infection, pregnancy — ASH 2019 Neunert; BSH 2024inputs: recent_drugs, autoimmune, recent_infection, pregnancyadvance: Trigger review complete
- 4RED_FLAGSMajor bleeding (ICH/GI) + plt <10k → STAT IVIG + steroids + platelet transfusion + TXA + emergency Rx — ASH 2019 Neunert; BSH 2024inputs: platelet_countadvance: Emergency bleeding stabilized
- 5INITIAL_WORKUPCBC + diff + smear + retic; coag; HIV / HCV / H. pylori; quantitative immunoglobulins; ANA; DAT (Evans) — ASH 2019 Neunert; BSH 2024inputs: cbc_with_diff, peripheral_smear, coag_panelactions: panel.cbc, panel.coag, workup.itp_standaloneadvance: Secondary causes excluded
- 6BRANCHING_WORKUPBone marrow biopsy if atypical (age >60, refractory, other cytopenias); 4Ts if heparin; ADAMTS13 if MAHA suspected — ASH 2019 Neunert; BSH 2024actions: calc.4tsadvance: Atypical features resolved
- 7DIFFERENTIALPrimary ITP / secondary (drug, autoimmune, viral, post-transfusion, vaccine-induced) / Evans / MDS / TTP / HIT / DIC — ASH 2019 Neunert; Provan 2019advance: Subtype assigned
- 8RISK_STRATIFICATIONITP-BAT bleeding score; thrombosis risk for TPO-RA; pregnancy management tier — ASH 2019 Neunert; Provan 2019advance: Risk documented
- 9TREATMENTPlt ≥30k + no bleeding → observe. Plt <30k or bleeding → dexamethasone 40 mg ×4d or prednisone 1 mg/kg; IVIG 1 g/kg ×1–2d for rapid response; major bleed → IVIG + IV methylpred + plt transfusion + TXA. Second-line: TPO-RA (romiplostim/eltrombopag/avatrombopag); rituximab; fostamatinib. Splenectomy reserved. — ASH 2019 Neunert; Provan 2019; Bussel 2021inputs: platelet_countadvance: Plan executed; if chronic → route to heme.itp.chronic.v1
- 10DISPOSITIONInpatient if plt <20k + bleeding; ICU if major bleed; outpatient otherwise with hem follow-up — ASH 2019 Neunert; BSH 2024advance: Disposition set
- 11MONITORINGPlt count q24–72h while titrating; LFTs on eltrombopag/fostamatinib; thrombosis surveillance on TPO-RA; steroid toxicity surveillance — ASH 2019 Neunert; NICE 2024inputs: platelet_countactions: panel.cbcadvance: Monitoring schedule documented
- 12FOLLOWUPHem clinic follow-up; vaccination plan if rituximab/splenectomy planned; transition to chronic engine if persistent >12 months — ASH 2019 Neunert; BSH 2024advance: Follow-up scheduled