Maternal-Fetal Medicine
HELLP Syndrome
HELLP syndrome requires rapid confirmation of microangiopathic hemolysis, hepatic injury, and thrombocytopenia; stabilization of severe hypertension and seizure risk; exclusion of competing thrombotic microangiopathies and acute fatty liver; and delivery when maternal or fetal status warrants.
Immediate decisions
Recognize HELLP and identify patients requiring urgent delivery
Triage simultaneously for maternal end-organ injury, hemorrhagic complications, and fetal compromise.
Suspect HELLP in a patient at 28 to 36 weeks' gestation or within 7 days postpartum who has right-upper-quadrant or epigastric pain, nausea or vomiting, headache, malaise, hypertension, proteinuria, or otherwise unexplained thrombocytopenia and transaminitis. Hypertension and proteinuria occur in up to 85% of cases, so their absence does not exclude HELLP. ScienceDirect+2ScienceDirectThe interpretation of liver function tests in pregnancyPubMedManaging hepatic complications of pregnancy: practical strategies for clinicians - PMCPubMedHELLP Syndrome - StatPearls - NCBI Bookshelf
Escalate immediately to obstetrics/MFM and a delivery-capable setting when there is eclampsia, severe hypertension, severe epigastric pain, pulmonary edema, worsening renal dysfunction, major maternal organ morbidity, or nonreassuring fetal status. These findings favor delivery over continued observation because HELLP is a severe hypertensive disorder with risk of progression and maternal morbidity. BMJ+1BMJHELLP syndrome - Management Approach | BMJ Best PracticePubMedPrevention, Diagnosis and Management of Hypertensive Disorders of Pregnancy: A Comparison of International Guidelines - PMC
Evaluate acute right-upper-quadrant pain with particular urgency when accompanied by hemodynamic instability, falling hemoglobin, or rapidly worsening liver tests, because HELLP may be complicated by hepatic hematoma, infarction, or rupture. Placental abruption should be considered with abdominal pain and a firm, painful uterus; abruption occurs in up to 10% of preeclamptic pregnancies. ScienceDirect+1ScienceDirectEclampsia and Preeclampsia - an overviewajogUnderstanding and managing HELLP syndrome: The integral role of aggressive glucocorticoids for mother and child - American Journal of Obstetrics & Gynecology
Obtain continuous or frequent maternal blood pressure assessment and fetal assessment while diagnostic laboratories are pending. PubMedPubMedPrevention, Diagnosis and Management of Hypertensive Disorders of Pregnancy: A Comparison of International Guidelines - PMC
Assess for neurologic symptoms, visual changes, dyspnea or hypoxemia, pulmonary edema, severe abdominal pain, vaginal bleeding, and uterine tenderness at presentation and with every clinical deterioration. PubMed+1PubMedPrevention, Diagnosis and Management of Hypertensive Disorders of Pregnancy: A Comparison of International Guidelines - PMCPubMedHELLP Syndrome - StatPearls - NCBI Bookshelf
Treat new generalized tonic-clonic seizure in this setting as eclampsia while evaluating alternative neurologic causes. PubMed+1PubMedACOG and local diagnostic criteria for hypertensive disorders of pregnancy (HDP) in La Paz-El Alto, Bolivia: A retrospective case-control studyPubMedHypertension In Pregnancy - StatPearls - NCBI Bookshelf
Initial workup
Confirm the HELLP laboratory pattern and establish severity
Order hemolysis, platelet, hepatic, renal, and coagulation studies together; trends matter when disease is evolving.
Obtain CBC with platelet count, peripheral blood smear, AST, ALT, total and indirect bilirubin, LDH, serum creatinine, and urine protein assessment in any patient with suspected HELLP. Confirm microangiopathic hemolysis with schistocytes or burr cells on smear and supportive evidence such as LDH greater than 600 IU/L, indirect hyperbilirubinemia, or low haptoglobin. ScienceDirect+2ScienceDirectEclampsia and Preeclampsia - an overviewPubMedHELLP Syndrome - StatPearls - NCBI BookshelfNeurologyClinical Reasoning: A 32-Year-Old Woman Presenting With Thunderclap Headache in the Third Trimester
Use the full triad for a firm diagnosis: platelet count below 100,000/μL, AST or ALT greater than twice the upper limit of normal, and hemolysis. Commonly used supporting thresholds include AST at least 70 IU/L, LDH above 600 IU/L, and total bilirubin above 1.2 mg/dL. Differences among Mississippi, Tennessee, and ACOG-derived criteria mean that a patient with evolving or partial disease warrants serial reassessment rather than exclusion solely because one threshold is not met. ScienceDirect+2ScienceDirectEclampsia and Preeclampsia - an overviewPubMedHypertension In Pregnancy - StatPearls - NCBI BookshelfNeurologyClinical Reasoning: A 32-Year-Old Woman Presenting With Thunderclap Headache in the Third Trimester
Classify thrombocytopenia to communicate severity and anticipate hemorrhagic risk: Mississippi class I is platelet count below 50,000/μL, class II is 50,000 to 100,000/μL, and class III is 100,000 to 150,000/μL with hemolysis and elevated liver enzymes. Partial HELLP may progress to complete disease, particularly when severe hypertension, eclampsia, severe epigastric pain, or organ morbidity is present. BMJ+1BMJHELLP syndrome - Management Approach | BMJ Best PracticeNeurologyClinical Reasoning: A 32-Year-Old Woman Presenting With Thunderclap Headache in the Third Trimester
Add coagulation testing when bleeding, abruption, hepatic injury, or severe systemic illness is present. Disseminated intravascular coagulation is a recognized HELLP complication and may manifest with elevated fibrin degradation products. ScienceDirectScienceDirectThe interpretation of liver function tests in pregnancy
Repeat platelet count, AST/ALT, LDH, bilirubin, creatinine, and clinical assessment when symptoms worsen or results are borderline, because HELLP may evolve rapidly. BMJ+1BMJHELLP syndrome - Management Approach | BMJ Best PracticeScienceDirectThe interpretation of liver function tests in pregnancy
Interpret an elevated creatinine, platelet count below 100,000/μL, liver enzymes above 80 IU/L, severe hypertension, severe headache, visual symptoms, dyspnea, or epigastric pain as severe-feature findings in the hypertensive-disorder framework. PubMedPubMedACOG and local diagnostic criteria for hypertensive disorders of pregnancy (HDP) in La Paz-El Alto, Bolivia: A retrospective case-control study
Do not require proteinuria to evaluate for HELLP; a meaningful minority of affected patients lack hypertension or proteinuria. ScienceDirect+1ScienceDirectThe interpretation of liver function tests in pregnancyPubMedManaging hepatic complications of pregnancy: practical strategies for clinicians - PMC
Diagnostic branching
Separate HELLP from competing pregnancy-associated microangiopathies and liver disease
The most consequential diagnostic error is missing TTP or acute fatty liver of pregnancy while assuming all abnormalities are HELLP.
TTP can have the same core findings as HELLP: thrombocytopenia, microangiopathic hemolytic anemia, renal disease, and neurologic abnormalities. In preeclampsia-associated HELLP, hypertension, proteinuria, and edema tend to precede hematologic abnormalities; in TTP, hypertension and proteinuria may follow hematologic disease. When thrombocytopenia and hemolysis are disproportionate to hepatic injury, or neurologic and renal findings dominate, involve hematology urgently and evaluate for TTP rather than relying on delivery as the sole intervention. ScienceDirect+1ScienceDirectEclampsia and Preeclampsia - an overviewScienceDirectHELLP Syndrome Developing at 14 Weeks of Gestation
Acute fatty liver of pregnancy is a key competing diagnosis in late pregnancy because it may overlap with HELLP in nausea, vomiting, liver dysfunction, thrombocytopenia, and systemic illness. Evaluate glucose, renal function, bilirubin, and coagulation status alongside HELLP laboratories when acute liver failure physiology or coagulopathy is present; AFLP and HELLP have similar clinical and laboratory presentations and require deliberate distinction. ScienceDirect+1ScienceDirectHELLP Syndrome Developing at 14 Weeks of GestationScienceDirectReview Imaging of pregnancy-related liver diseases
Consider antiphospholipid syndrome and hemolytic uremic syndrome in pregnancy-associated thrombotic microangiopathy, particularly when the timing, renal injury, thrombotic history, or laboratory pattern is atypical for hypertensive disease. These entities are specifically recognized in the differential diagnosis of HELLP. ScienceDirectScienceDirectHELLP Syndrome Developing at 14 Weeks of Gestation
Do not label isolated pruritus and cholestatic liver-test elevation as HELLP without evidence of hemolysis and thrombocytopenia. Intrahepatic cholestasis of pregnancy is a diagnosis of exclusion, and ursodeoxycholic acid is first-line therapy at 10 to 15 mg/kg maternal body weight when ICP is diagnosed. PubMedPubMedManaging hepatic complications of pregnancy: practical strategies for clinicians - PMC
Favor HELLP when hemolysis, elevated transaminases, and platelet count below 100,000/μL occur with a hypertensive-disorder phenotype, but continue reassessment if the trajectory is atypical. ScienceDirect+1ScienceDirectEclampsia and Preeclampsia - an overviewPubMedHypertension In Pregnancy - StatPearls - NCBI Bookshelf
Escalate suspected TTP to hematology urgently because it shares the HELLP laboratory phenotype but has a separate treatment pathway. ScienceDirect+1ScienceDirectEclampsia and Preeclampsia - an overviewScienceDirectHELLP Syndrome Developing at 14 Weeks of Gestation
Obtain targeted imaging for suspected hepatic hematoma, infarction, or rupture when severe right-upper-quadrant pain is accompanied by instability or evidence of bleeding. ajog+1ajogUnderstanding and managing HELLP syndrome: The integral role of aggressive glucocorticoids for mother and child - American Journal of Obstetrics & GynecologyScienceDirectExtensive hepatic infarction in severe preeclampsia as part ...
| Condition | Pattern that changes the differential | Action |
|---|---|---|
| HELLP syndrome | Hemolysis, platelet count <100,000/μL, and transaminase elevation; hypertension and proteinuria are common but not universal. ScienceDirect+2ScienceDirectEclampsia and Preeclampsia - an overviewPubMedManaging hepatic complications of pregnancy: practical strategies for clinicians - PMCPubMedHypertension In Pregnancy - StatPearls - NCBI Bookshelf | Stabilize severe features, begin seizure prophylaxis when indicated, and plan delivery based on maternal-fetal status. PubMedPubMedPrevention, Diagnosis and Management of Hypertensive Disorders of Pregnancy: A Comparison of International Guidelines - PMC |
| Thrombotic thrombocytopenic purpura | May be indistinguishable by thrombocytopenia, hemolytic anemia, renal disease, and neurologic abnormalities; hypertension/proteinuria may follow hematologic findings. ScienceDirectScienceDirectEclampsia and Preeclampsia - an overview | Urgent hematology involvement and TTP-directed evaluation; do not assume delivery alone addresses the process. ScienceDirect+1ScienceDirectEclampsia and Preeclampsia - an overviewScienceDirectHELLP Syndrome Developing at 14 Weeks of Gestation |
| Acute fatty liver of pregnancy | Overlaps with HELLP; assess for acute liver failure physiology, hypoglycemia, renal dysfunction, and coagulopathy. ScienceDirect+1ScienceDirectHELLP Syndrome Developing at 14 Weeks of GestationScienceDirectReview Imaging of pregnancy-related liver diseases | Urgent obstetric and critical-care assessment with delivery planning and management of hepatic failure complications. ScienceDirectScienceDirectReview Imaging of pregnancy-related liver diseases |
| Hemolytic uremic syndrome or antiphospholipid syndrome | Pregnancy-associated thrombotic microangiopathy or thrombotic phenotype atypical for HELLP. ScienceDirectScienceDirectHELLP Syndrome Developing at 14 Weeks of Gestation | Obtain specialist-directed evaluation and treat the identified disorder rather than applying a HELLP-only pathway. ScienceDirectScienceDirectHELLP Syndrome Developing at 14 Weeks of Gestation |
| Intrahepatic cholestasis of pregnancy | Cholestatic liver disease without the defining HELLP hemolysis-thrombocytopenia pattern. PubMedPubMedManaging hepatic complications of pregnancy: practical strategies for clinicians - PMC | Exclude other causes; use ursodeoxycholic acid 10–15 mg/kg when ICP is diagnosed. PubMedPubMedManaging hepatic complications of pregnancy: practical strategies for clinicians - PMC |
Maternal stabilization
Stabilize severe features while preparing definitive obstetric management
Delivery is definitive treatment for the hypertensive disorder; stabilization prevents stroke, seizure, and cardiopulmonary deterioration during that process.
Manage HELLP as preeclampsia with severe features: stabilize blood pressure, prevent eclampsia, assess volume status and pulmonary edema, correct clinically significant hemorrhagic complications, and coordinate anesthesia, obstetric, neonatal, and critical-care planning according to maternal and fetal condition. Definitive treatment of preeclampsia-spectrum disease is delivery. PubMed+2PubMedPrevention, Diagnosis and Management of Hypertensive Disorders of Pregnancy: A Comparison of International Guidelines - PMCPubMedHypertension In Pregnancy - StatPearls - NCBI BookshelfPubMedPreeclampsia - StatPearls - NCBI Bookshelf
Administer magnesium sulfate for seizure prophylaxis in women with preeclampsia with severe features during labor and delivery and continue for 24 hours postpartum. Use this pathway in HELLP because HELLP is managed as a severe preeclampsia phenotype and eclampsia can occur intrapartum or postpartum. PubMed+2PubMedPrevention, Diagnosis and Management of Hypertensive Disorders of Pregnancy: A Comparison of International Guidelines - PMCPubMedHypertension In Pregnancy - StatPearls - NCBI BookshelfPubMedPlasma exchange in severe postpartum HELLP syndrome
Treat severe hypertension pharmacologically without delay; intensive antihypertensive treatment is used to minimize severe systolic hypertension and its associated stroke risk in HELLP. Select the agent based on clinician experience, cost, and local availability; the cited guideline comparison does not establish a single preferred medication or dose. BMJ+1BMJHELLP syndrome - Management Approach | BMJ Best PracticePubMedPrevention, Diagnosis and Management of Hypertensive Disorders of Pregnancy: A Comparison of International Guidelines - PMC
Do not use corticosteroids as maternal disease-directed treatment for HELLP outside an individualized specialist plan. Guideline comparison data specifically advise against corticosteroids for treating HELLP, despite older literature describing possible maternal-fetal benefit; this disagreement does not alter the need for maternal stabilization and delivery when indicated. PubMed+1PubMedPrevention, Diagnosis and Management of Hypertensive Disorders of Pregnancy: A Comparison of International Guidelines - PMCajogUnderstanding and managing HELLP syndrome: The integral role of aggressive glucocorticoids for mother and child - American Journal of Obstetrics & Gynecology
Use delivery planning immediately for HELLP with uncontrolled severe-range blood pressure, persistent headache or upper abdominal pain, visual symptoms, stroke, myocardial infarction, worsening renal function, pulmonary edema, eclampsia, or abnormal fetal testing. PubMedPubMedPrevention, Diagnosis and Management of Hypertensive Disorders of Pregnancy: A Comparison of International Guidelines - PMC
Involve anesthesia early when thrombocytopenia is substantial or hemorrhage and urgent operative delivery are plausible; platelet trajectory and coagulation status should inform procedural planning. ScienceDirect+1ScienceDirectThe interpretation of liver function tests in pregnancyNeurologyClinical Reasoning: A 32-Year-Old Woman Presenting With Thunderclap Headache in the Third Trimester
Monitor for pulmonary edema and avoid uncritical volume loading; volume status is a recognized component of acute supportive management. PubMedPubMedHELLP Syndrome - StatPearls - NCBI Bookshelf
Delivery timing and expectant management
HELLP syndrome itself is an earlier-delivery indication in guideline comparisons of hypertensive disorders of pregnancy. Continued observation may be considered only when maternal and fetal status are stable in a setting capable of serial maternal laboratory testing, blood-pressure treatment, fetal surveillance, and immediate delivery; instability overrides gestational-age considerations. PubMed+1PubMedPrevention, Diagnosis and Management of Hypertensive Disorders of Pregnancy: A Comparison of International Guidelines - PMCPubMedPreeclampsia - StatPearls - NCBI Bookshelf
At any gestational age, class III or partial HELLP accompanied by eclampsia, severe epigastric pain, severe hypertension, or major organ-system morbidity should be treated as severe disease requiring escalation and delivery planning. BMJBMJHELLP syndrome - Management Approach | BMJ Best Practice
After delivery
Continue surveillance postpartum and reconsider the diagnosis if recovery is atypical
Delivery ends the pregnancy trigger but does not eliminate immediate postpartum risk.
Continue inpatient clinical and laboratory surveillance after delivery because HELLP can develop during the first postpartum week and eclampsia most often occurs from intrapartum through 72 hours postpartum. Follow blood pressure, symptoms, platelet count, hemolysis markers, liver tests, renal function, respiratory status, and bleeding risk until the trajectory is clearly improving. ScienceDirect+2ScienceDirectThe interpretation of liver function tests in pregnancyPubMedManaging hepatic complications of pregnancy: practical strategies for clinicians - PMCPubMedHypertension In Pregnancy - StatPearls - NCBI Bookshelf
Continue magnesium sulfate for 24 hours postpartum when the patient meets the severe-feature prophylaxis pathway. Treat postpartum severe hypertension pharmacologically; international guideline comparisons endorse treatment of severe hypertension during pregnancy and postpartum. PubMedPubMedPrevention, Diagnosis and Management of Hypertensive Disorders of Pregnancy: A Comparison of International Guidelines - PMC
If platelet count, hemolysis, renal injury, neurologic dysfunction, or hepatic abnormalities fail to improve as expected after delivery, reopen the differential rather than assuming persistent HELLP. TTP, hemolytic uremic syndrome, antiphospholipid syndrome, and acute fatty liver of pregnancy are competing diagnoses that require disease-specific evaluation and management. ScienceDirect+2ScienceDirectEclampsia and Preeclampsia - an overviewScienceDirectHELLP Syndrome Developing at 14 Weeks of GestationScienceDirectReview Imaging of pregnancy-related liver diseases
Arrange postpartum support and counseling after a severe hypertensive pregnancy complication; affected patients and families may have substantial psychological distress, and postpartum management should include psychological and family support. PubMedPubMedPrevention, Diagnosis and Management of Hypertensive Disorders of Pregnancy: A Comparison of International Guidelines - PMC
Reassess urgently for headache, visual symptoms, seizure, dyspnea, severe hypertension, recurrent epigastric pain, bleeding, or reduced urine output after delivery. PubMed+2PubMedPrevention, Diagnosis and Management of Hypertensive Disorders of Pregnancy: A Comparison of International Guidelines - PMCPubMedHELLP Syndrome - StatPearls - NCBI BookshelfPubMedHypertension In Pregnancy - StatPearls - NCBI Bookshelf
Do not discontinue diagnostic vigilance solely because delivery has occurred; postpartum-onset HELLP remains possible for up to 7 days. PubMed+1PubMedManaging hepatic complications of pregnancy: practical strategies for clinicians - PMCPubMedHELLP Syndrome - StatPearls - NCBI Bookshelf
Escalate persistent thrombotic microangiopathy features to hematology and nephrology as clinically indicated. ScienceDirect+1ScienceDirectEclampsia and Preeclampsia - an overviewScienceDirectHELLP Syndrome Developing at 14 Weeks of Gestation
References
- HELLP syndrome - Management Approach | BMJ Best Practice — bestpractice.bmj.com · bestpractice.bmj.com
- Eclampsia and Preeclampsia - an overview — www.sciencedirect.com · www.sciencedirect.com
- The interpretation of liver function tests in pregnancy — www.sciencedirect.com · www.sciencedirect.com
- Clinical diagnosis and treatment of “atypical” HELLP ... — www.sciencedirect.com · www.sciencedirect.com
- HELLP Syndrome Developing at 14 Weeks of Gestation — www.sciencedirect.com · www.sciencedirect.com
- Prevention, Diagnosis and Management of Hypertensive Disorders of Pregnancy: A Comparison of International Guidelines - PMC — pmc.ncbi.nlm.nih.gov · pmc.ncbi.nlm.nih.gov
- Understanding and managing HELLP syndrome: The integral role of aggressive glucocorticoids for mother and child - American Journal of Obstetrics & Gynecology — www.ajog.org · www.ajog.org
- The HELLP syndrome: Clinical issues and management. A Review — pmc.ncbi.nlm.nih.gov · pmc.ncbi.nlm.nih.gov
- Managing hepatic complications of pregnancy: practical strategies for clinicians - PMC — pmc.ncbi.nlm.nih.gov · pmc.ncbi.nlm.nih.gov
- ACOG and local diagnostic criteria for hypertensive disorders of pregnancy (HDP) in La Paz-El Alto, Bolivia: A retrospective case-control study — pmc.ncbi.nlm.nih.gov · pmc.ncbi.nlm.nih.gov
- HELLP Syndrome - StatPearls - NCBI Bookshelf — www.ncbi.nlm.nih.gov · www.ncbi.nlm.nih.gov
- Hypertension In Pregnancy - StatPearls - NCBI Bookshelf — www.ncbi.nlm.nih.gov · www.ncbi.nlm.nih.gov
- Preeclampsia - StatPearls - NCBI Bookshelf — www.ncbi.nlm.nih.gov · www.ncbi.nlm.nih.gov
- Clinical Reasoning: A 32-Year-Old Woman Presenting With Thunderclap Headache in the Third Trimester — www.neurology.org · www.neurology.org
- Plasma exchange in severe postpartum HELLP syndrome — pubmed.ncbi.nlm.nih.gov · pubmed.ncbi.nlm.nih.gov
- Table B-1, Excluded articles and records with reasons for exclusion - Management of Postpartum Hypertensive Disorders of Pregnancy - NCBI Bookshelf — www.ncbi.nlm.nih.gov · www.ncbi.nlm.nih.gov
- Preeclampsia: Pathophysiology, Management, and ... — publications.aap.org · publications.aap.org
- Revisiting HELLP syndrome — www.sciencedirect.com · www.sciencedirect.com
- HELLP Syndrome - an overview — www.sciencedirect.com · www.sciencedirect.com
- Extensive hepatic infarction in severe preeclampsia as part ... — www.sciencedirect.com · www.sciencedirect.com
- Review Imaging of pregnancy-related liver diseases — www.sciencedirect.com · www.sciencedirect.com
- A life-saving therapy in Class I HELLP syndrome — www.sciencedirect.com · www.sciencedirect.com
- Direct evidence of complement activation in HELLP ... — www.sciencedirect.com · www.sciencedirect.com
- Does the hepatologist still need to rely on ... — www.sciencedirect.com · www.sciencedirect.com