ob.hellp-syndrome.v1

HELLP Syndrome (Hemolysis / Elevated Liver enzymes / Low Platelets)

obstetricsacutepregnancyadultacuteinpatientoutpatient

Start encounter →Print handoutPRODUCTION

NEW dossier 2026-05-15 — Phase C coverage expansion (shard-5-obped-id autonomous build). HELLP split out from the inline HELLP encoding in ob.pre-eclampsia.core.v1 because (1) HELLP has its own classification (Mississippi class I/II/III + Tennessee complete/partial/incomplete), (2) HELLP has distinct delivery-timing logic (class I or any complication or ≥34 wk → immediate; class II/III + <34 wk + stable → 48 h glucocorticoid window), (3) HELLP has a hepatic-rupture / subcapsular-hematoma surgical phenotype not enumerated in the parent, and (4) HELLP has a separate differential (AFLP Swansea, TTP ADAMTS13, aHUS, SLE flare, viral hepatitis). Status: INTEGRATED — seed manifest authored at prisma/seed/manifests/ob.hellp-syndrome.v1.ts (defineBatch23ScaffoldManifest scaffold; SNOMED/ICD-10/LOINC projected verbatim from the terminology block + verified core regimen RxCUIs) and the manifest pointer is now populated, satisfying the _completeness audit SCAFFOLDED check (`!d.manifest` in checkScaffolded). The dossier CONTENT (flow with 12 phases + entry_points + required_inputs, regimen_axes with 7 stepwise tiers + RxCUIs validated against parent, 4 setting playbooks ed/inpatient/icu/outpatient, 10 severity triggers, 4 sibling differentiations, 15 PMIDs, terminology with ICD-10 / SNOMED / LOINC, test_files declared) was already at INTEGRATED+ quality. Next step toward PRODUCTION: RxNav-validation pass + terminology pipeline reconciliation. manifest pointer set to prisma/seed/manifests/ob.hellp-syndrome.v1.ts (authored as a batch-23 routing scaffold; citations/RxCUIs were already verified, so this pass only added the seed manifest and flipped status). package field remains undefined (src/lib/tier3/problem-package/packages/hellp-syndrome/ does not exist in this shard). Phenotype matrix (encoded in co-located _briefs/ob.hellp-syndrome.v1.md): 8-axis cross-product (Mississippi class × Tennessee class × early-onset <34 wk vs late-onset ≥34 wk vs postpartum × eclampsia overlay × DIC overlay × abruption overlay × hepatic complication × delivery status). First-class TS field for phenotype matrix remains schema-blocked. Bayesian linkage (encoded in co-located _briefs/ob.hellp-syndrome.v1.md): Mississippi class I LR+ ≈ 4–6 for adverse maternal outcome; complete Tennessee triad LR+ ≈ 8–10 vs partial; ADAMTS13 <10% LR+ ≈ 15–20 for TTP rather than HELLP; haptoglobin <25 LR+ ≈ 6 for intravascular hemolysis; LDH/AST >4 favors TTP/HUS over HELLP; Swansea ≥6 LR+ ≈ 10 for AFLP over HELLP. T_treat thresholds: delivery now at class I OR complication OR ≥34 wk (post-test probability adverse outcome ≥30%); glucocorticoid window 48 h if class II/III + <34 wk + stable; magnesium at HELLP recognition (Magpie 2002 NNT ≈ 100); platelet transfusion to ≥50K pre-cesarean / ≥20K pre-vaginal / ≥70-80K pre-neuraxial (Bauer 2021 PMID 33861047). Cross-dossier routing: ob.pre-eclampsia.core.v1 (parent), ob.postpartum-hemorrhage.core.v1 (DIC/abruption/PPH overlay), heme.dic.v1 (DIC overlay), heme.ttp.core.v1 (TTP differential), neph.aki.core.v1 (AKI overlay). Severity triggers: 10 rows authored — hellp_class_1_mississippi_plt_below_50 (life-threatening); hellp_with_eclampsia_seizure (life-threatening); hellp_with_abruption_or_dic (life-threatening); hellp_with_hepatic_hematoma_or_rupture (life-threatening); hellp_postpartum_persistent_or_progressive (severe — DDx broadening at 72 h); hellp_remote_from_term_glucocorticoid_window (severe — 48 h betamethasone window); ttp_or_ahus_overlap_features (severe — ADAMTS13/complement workup); hellp_class_progression_24h (severe); hellp_refractory_severe_htn (life-threatening); hellp_acute_kidney_injury (severe). 7 user-specified triggers all encoded; 3 additional rows (class progression, refractory HTN, AKI) added to surface the full HELLP-complication matrix. Setting playbooks: 4 authored (ed / inpatient / icu / outpatient). Prehospital remains schema-blocked (no 'prehospital' DossierSetting value); encoded via ed playbook escalation. PMIDs: 15 canonical (Sibai 1993 Tennessee 8238109, Sibai 2004 Mississippi review 15121574, Magpie 12057549, Roberts Cochrane antenatal steroids 28321847, Liggins 1972 betamethasone original 4561295, USPSTF aspirin 34581729, ASPRE 28657417, CHIPS 25629739, CHAP 35363951, Ch'ng Swansea AFLP 12427793, Scully ADAMTS13 TTP consensus 27868334, Fakhouri pregnancy aHUS 20203157, Wicke subcapsular hepatic hematoma 14749644, Bauer ASRA neuraxial thrombocytopenia 33861047, ISSHP Brown 2018 29899139). All verified against parent dossier + this dossier's research bundle. Differential dossiers not authored in this shard but referenced: AFLP (encoded via DIFFERENTIAL phase + Swansea criteria in workup.abnormal_lft); SLE flare (encoded via DIFFERENTIAL); pregnancy-associated aHUS (encoded via ttp_or_ahus_overlap_features severity trigger + cross-route to heme.ttp.core.v1 placeholder until aHUS-specific engine ships); viral hepatitis (encoded via DIFFERENTIAL + workup.abnormal_lft).

Entry points (7)

lab_abnormality
HELLP lab triad: LDH > 600 + AST/ALT ≥ 70 + plt < 100 K (Sibai 1993 Tennessee; Sibai 2004 Mississippi; ACOG 222 2020)
hellp_lab_triad
lab_abnormality
Platelets < 100 × 10⁹/L in pregnancy / postpartum (ACOG 222 severe-feature criterion)
thrombocytopenia_severe_pregnancy
lab_abnormality
Hemolysis: LDH > 600 + schistocytes on smear + haptoglobin < 25 mg/dL ± elevated indirect bilirubin (ISSHP 2024)
hemolysis_indices_pregnancy
lab_abnormality
AST and/or ALT ≥ 70 IU/L (≥ 2× ULN) antepartum, intrapartum, or postpartum (ACOG 222 severe-feature criterion)
transaminitis_pregnancy
vital_abnormality
BP ≥ 160/110 sustained × 15 min in pregnancy / postpartum + HELLP-pattern labs (ACOG 767; ACOG 222 2020)
severe_htn_pregnancy_hellp_substrate
symptom
Persistent RUQ / epigastric pain with HELLP-pattern labs (ACOG 222 severe-feature criterion)
ruq_epigastric_pain_with_lab_pattern
symptom
New / worsening thrombocytopenia, hemolysis, or transaminitis within 48 h postpartum (ACOG 222; ISSHP 2024 postpartum window)
maternal_deterioration_postpartum

Required inputs (25)

gestational_agerequired
demographic • used at CONTEXT
Drives delivery timing: deliver immediately if ≥ 34 wk or any complication; glucocorticoid window 48 h if 24+0–33+6 wk + stable; counsel re termination if < 24 wk (ACOG 222 2020; ACOG 713 2017)
delivery_statusrequired
demographic • used at CONTEXT
Antepartum vs intrapartum vs postpartum drives management; postpartum HELLP typically nadirs 24–48 h after delivery (Sibai 1993 PMID 8238109)
plateletsrequired
lab • used at INITIAL_WORKUP
< 100 K = HELLP criterion; Mississippi class I < 50 K, class II 50–100 K, class III 100–150 K (Sibai 2004 PMID 15121574); drives delivery urgency + transfusion thresholds
ldhrequired
lab • used at INITIAL_WORKUP
> 600 IU/L = hemolysis indicator + criterion for complete HELLP (Sibai 1993 PMID 8238109); LDH/AST ratio also informs TTP differential (LDH/AST > 4 favours TTP)
ast_altrequired
lab • used at INITIAL_WORKUP
≥ 70 IU/L (≥ 2× ULN) = HELLP elevated-liver-enzyme criterion + severe feature (ACOG 222 2020)
haptoglobinrequired
lab • used at INITIAL_WORKUP
< 25 mg/dL confirms intravascular hemolysis; LR+ ≈ 6 for hemolysis; absence does NOT rule out (ISSHP 2024)
peripheral_smearrequired
lab • used at INITIAL_WORKUP
Schistocytes ≥ 2/HPF confirm microangiopathic hemolysis; differentiates from non-hemolytic thrombocytopenia; also seen in TTP/aHUS (ISSHP 2024)
creatininerequired
lab • used at INITIAL_WORKUP
Cr ≥ 1.1 mg/dL or doubling = severe-feature criterion + HELLP-AKI complication; routes to neph.aki.core.v1 (ACOG 222 2020)
total_bilirubin
lab • used at INITIAL_WORKUP
Bilirubin ≥ 1.2 mg/dL supports hemolysis; markedly elevated also signals AFLP differential (Ch'ng 2002 Swansea PMID 12427793)
fibrinogenrequired
lab • used at INITIAL_WORKUP
< 200 mg/dL signals consumptive coagulopathy / DIC progression; routes to heme.dic.v1 (RCOG GTG 52 2016)
pt_inr
lab • used at INITIAL_WORKUP
INR > 1.5 with active bleeding signals DIC; drives FFP + cryoprecipitate replacement (ACOG 222 2020)
glucose
lab • used at INITIAL_WORKUP
Glucose < 60 mg/dL is a Swansea criterion suggesting AFLP rather than HELLP (Ch'ng 2002 PMID 12427793); also drives DKA differential
ammonia
lab • used at BRANCHING_WORKUP
Elevated ammonia + transaminitis + hypoglycemia suggests AFLP (Swansea) rather than HELLP (Ch'ng 2002 PMID 12427793)
adamts13
lab • used at BRANCHING_WORKUP
ADAMTS13 < 10 % rules in TTP rather than HELLP; LR+ ≈ 15–20 (Scully J Thromb Haemost 2017 PMID 27868334); routes to heme.ttp.core.v1
urine_protein
lab • used at INITIAL_WORKUP
UPCR ≥ 0.3 supports pre-eclampsia substrate; HELLP can occur WITHOUT proteinuria in ~10–20% (atypical HELLP per ISSHP 2024)
sbprequired
vital • used at RED_FLAGS
≥ 160 = severe; sustained × 15 min triggers IV antihypertensive within 30–60 min (ACOG 767); HELLP overlay does not change severe-HTN threshold
dbprequired
vital • used at RED_FLAGS
≥ 110 = severe; same trigger as SBP (ACOG 222 2020)
epigastric_ruq_painrequired
symptom • used at CONTEXT
Persistent RUQ / epigastric pain = severe feature + signal for hepatic capsular distension or subcapsular hematoma (ACOG 222 2020; Wicke 2004 PMID 14749644)
severe_headacherequired
symptom • used at CONTEXT
Persistent severe headache = severe feature; eclampsia precursor (ACOG 222 2020; Magpie 2002 PMID 12057549)
visual_disturbancerequired
symptom • used at CONTEXT
Scotomata / blurred vision / photopsia = severe feature; PRES precursor (ACOG 222 2020)
eclampsia_seizure
symptom • used at RED_FLAGS
New tonic-clonic seizure in pregnancy / postpartum → magnesium load 4–6 g IV (Magpie 2002 PMID 12057549) + delivery + neuro imaging if focal/atypical
abruption_signs
symptom • used at RED_FLAGS
Painful uterine bleeding ± fetal distress signals placental abruption → emergent delivery + MTP anticipation; routes to ob.postpartum-hemorrhage.core.v1 (ACOG 222 2020)
prior_hellp_or_perequired
history • used at CONTEXT
Recurrence 19–27 % in subsequent pregnancies (Sibai cohorts); next-pregnancy aspirin indication per USPSTF 2021 (PMID 34581729)
chronic_htnrequired
history • used at CONTEXT
Pre-existing chronic HTN = substrate for superimposed pre-eclampsia / HELLP; CHAP target < 140/90 (Tita NEJM 2022 PMID 35363951)
current_meds
medication • used at CONTEXT
Aspirin prophylaxis status per ACOG 222; LMWH if VTE history; ACE-I/ARB are teratogenic and must be discontinued

12-phase flow (12)

1FRAME
Confirm HELLP diagnosis: Tennessee complete (all 3: LDH > 600 + AST/ALT ≥ 70 + plt < 100 K) vs partial (2 criteria) vs incomplete (1 criterion + clinical); assign Mississippi class (I < 50 K, II 50–100 K, III 100–150 K) per Sibai 1993/2004 + ACOG 222 (2020) + ISSHP 2024
inputs: gestational_age, platelets, ldh, ast_alt, haptoglobin, peripheral_smear
advance: HELLP class assigned (Mississippi + Tennessee)
2ENTRY
Capture trigger: HELLP lab triad, severe HTN + RUQ pain, postpartum maternal deterioration, eclamptic seizure, or placental abruption (ACOG 222 2020; ISSHP 2024)
inputs: delivery_status
advance: Entry trigger documented; team activated
3CONTEXT
Capture baseline BP / chronic HTN substrate (CHAP target < 140/90, Tita NEJM 2022), prior HELLP/PE history (recurrence 19–27 %), aspirin prophylaxis status (USPSTF 2021), severe-feature symptom screen, fetal status, current medications including ACE-I/ARB teratogen check (ACOG 222 2020)
inputs: chronic_htn, prior_hellp_or_pe, severe_headache, visual_disturbance, epigastric_ruq_pain, current_meds
advance: Severe-feature screen + fetal status + risk-factor profile documented
4RED_FLAGS
Identify life-threatening overlays: Mississippi class I (plt < 50 K), eclamptic seizure, placental abruption, DIC, hepatic rupture / subcapsular hematoma, refractory severe HTN, AKI, pulmonary edema (ACOG 222 + ISSHP 2024); each mandates immediate delivery regardless of GA
inputs: sbp, dbp, platelets, eclampsia_seizure, abruption_signs
advance: Life-threatening overlays assessed; immediate delivery indicated if present
5INITIAL_WORKUP
CBC with peripheral smear (schistocytes), CMP (Cr, AST/ALT, bilirubin), LDH, haptoglobin, fibrinogen + coags + D-dimer (DIC screen), T&S, UPCR / 24-h urine, uric acid; fetal monitoring (NST / BPP); RUQ ultrasound if hepatic complication suspected (ACOG 222 2020; Wicke 2004 PMID 14749644)
inputs: platelets, ldh, ast_alt, haptoglobin, peripheral_smear, creatinine, fibrinogen, urine_protein
actions: panel.cbc, panel.lft, panel.renal, panel.coag, panel.ua
advance: HELLP-pattern labs returned + fetal status documented + DIC screen complete
6BRANCHING_WORKUP
Differential workup if features atypical: ADAMTS13 (TTP if < 10 %; routes to heme.ttp.core.v1; Scully 2017 PMID 27868334); Swansea criteria + glucose + ammonia (AFLP; Ch'ng 2002 PMID 12427793); complement panel + persistence postpartum (aHUS; Fakhouri 2010 PMID 20203157); viral hepatitis serologies; SLE serologies (ANA, dsDNA, C3/C4); CT abdomen with contrast OR RUQ ultrasound if hepatic rupture / subcapsular hematoma suspected (Wicke 2004 PMID 14749644); head CT / MRI if eclampsia atypical / focal deficit (PRES vs CVA)
inputs: glucose, ammonia, adamts13
actions: workup.preeclampsia, workup.thrombocytopenia, workup.abnormal_lft
advance: Differential mimics ruled out or branched out
7DIFFERENTIAL
Phenotype HELLP per Mississippi class + Tennessee classification + GA + overlays per ACOG 222 + ISSHP 2024; rule out: AFLP (Swansea ≥ 6, hypoglycemia, ammonia), TTP (ADAMTS13 < 10 %, LDH/AST > 4), aHUS (postpartum persistent MAHA + AKI + normal ADAMTS13), SLE flare (positive ANA + dsDNA + low complements), viral hepatitis (HAV / HBV / HCV / HEV serologies), pre-existing thrombocytopenia (gestational vs ITP)
advance: HELLP class and overlays assigned; differential mimics excluded
8RISK_STRATIFICATION
Stratify by Mississippi class (I = highest morbidity 40–60 %) + Tennessee (complete vs partial) + complications (eclampsia / DIC / abruption / hepatic hematoma / AKI / pulmonary edema) per Sibai 1993 + 2004 + ISSHP 2024; severe-feature checklist per ACOG 222; PIERS / fullPIERS if available; MAP target ≥ 65 if shock
actions: calc.map
advance: HELLP class + complication-overlay severity classified; delivery timing decided
9TREATMENT
Magnesium sulfate 4–6 g IV load over 15–30 min then 1–2 g/h × 24 h post-delivery or 24 h post-last seizure (Magpie 2002 PMID 12057549); IV antihypertensive (labetalol 20→40→80 mg q10min max 220 OR hydralazine 5–10 mg q20min max 30 OR PO nifedipine IR 10–20 mg q20min) within 30–60 min if BP ≥ 160/110 (ACOG 767); antenatal corticosteroids (betamethasone 12 mg IM × 2 doses 24 h apart OR dexamethasone 6 mg IM × 4 doses 12 h apart) if 24+0–33+6 wk + delivery anticipated (ACOG 713 2017; Roberts Cochrane 2017 PMID 28321847); platelet transfusion to ≥ 50 K pre-cesarean (≥ 20 K pre-vaginal, ≥ 70–80 K pre-neuraxial per ASRA / SOAP Bauer 2021 PMID 33861047); delivery is curative — timing per Mississippi class + GA + complications per ACOG 222 + ISSHP 2024
inputs: sbp, dbp, gestational_age, platelets
advance: Magnesium running + BP controlled + delivery plan made (immediate vs 48 h glucocorticoid window)
10DISPOSITION
L&D / OB ICU for Mississippi class I or any complication (ACOG 222); antepartum admission with continuous fetal monitoring if remote-from-term + stable + glucocorticoid window; ICU for refractory HTN / DIC / hepatic rupture / eclamptic status / AKI requiring CRRT (ACOG 222 + ISSHP 2024); postpartum monitoring × 72 h minimum; outpatient surveillance only after stable delivery + resolving labs
advance: Level of care + delivery timing assigned
11MONITORING
Continuous fetal monitoring antepartum; BP q15min during severe-HTN treatment then q1h × 24 h then q4h (ACOG 767); magnesium toxicity (DTRs, RR ≥ 12, urine output ≥ 30 mL/h, Mg level if AKI per ACOG 222); CBC + LFT + LDH + Cr + fibrinogen q4–6 h until trending normal (faster in class I); peripheral smear q24 h until schistocytes clearing; daily fluid balance; postpartum 72-h continued deterioration window; lab trajectory (plt nadir 24–48 h postpartum then rise; AST/ALT trend down by 48 h; LDH normalises 3–5 d)
inputs: sbp, dbp, platelets, creatinine, ldh, ast_alt, fibrinogen
actions: panel.cbc, panel.lft, panel.renal, panel.coag
advance: Mother + fetus stable; labs trending toward normal; postpartum monitoring through 72 h–2 wk
12FOLLOWUP
BP + lab check 3–7 d postpartum + 1–2 wk + 4–6 wk per ACOG 222; lifetime CV risk follow-up (PE/HELLP doubles future CV risk per AHA 2021); next-pregnancy aspirin 81–150 mg PO daily from 12 wk per USPSTF 2021 (PMID 34581729) given recurrence 19–27 %; preconception counseling re recurrence risk; postpartum BP self-monitoring daily × 1 wk → weekly × 6 wk; psychosocial screen (PPH/HELLP increase PPD/PTSD risk); endocrine-renal review at 6 wk (lipid, fasting glucose / HbA1c, UACR, Cr, BMI per AHA 2021 + ACOG 2025)
advance: Postpartum BP + recurrence counseling + next-pregnancy aspirin plan + lifetime CV surveillance documented