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Summary Table

Feature	Detail
Risk Factors	Multiparity (unlike preeclampsia), maternal age > 25, white ethnicity, prior history of HELLP or preeclampsia, chronic hypertension, antiphospholipid syndrome, obesity
Etiology	Endothelial dysfunction and microangiopathic process arising from abnormal placentation, representing a severe variant within the preeclampsia spectrum
Patient Presentation	Third-trimester pregnant woman (or early postpartum) with malaise, nausea/vomiting, and right upper quadrant or epigastric pain; may initially be misdiagnosed as gastritis, hepatitis, or gallbladder disease
Classic Physical Exam	RUQ tenderness, hepatomegaly (sometimes), hypertension (variable and may be absent), edema, jaundice (in severe hemolysis)
Key Diagnostic Results	Hemolysis (elevated LDH > 600 IU/L, schistocytes on peripheral smear, low haptoglobin, elevated indirect bilirubin), Elevated Liver enzymes (AST/ALT more than twice the upper limit of normal), Low Platelets (< 100,000/microL)
Management	Stabilize with magnesium sulfate (seizure prophylaxis), antihypertensives if BP is in the severe range, corticosteroids for fetal lung maturity if < 34 weeks, and delivery as definitive treatment
Keywords	"RUQ pain in pregnancy with thrombocytopenia," "schistocytes on peripheral smear in pregnant woman," "elevated LDH with low platelets in third trimester," "nausea and malaise misdiagnosed as viral illness in late pregnancy"

1. Pathophysiology

HELLP syndrome shares its origin with preeclampsia: defective trophoblastic invasion of the spiral arteries during early placentation. When cytotrophoblasts fail to adequately remodel these arteries, the placenta becomes chronically hypoperfused. In response, the ischemic placenta releases antiangiogenic factors (sFlt-1, soluble endoglin) and pro-inflammatory mediators into the maternal circulation, generating widespread endothelial injury.

What distinguishes HELLP from "ordinary" severe preeclampsia is the dominant expression of this endothelial damage in the hepatic and hematologic systems. The endothelial injury triggers diffuse platelet activation and aggregation at sites of damaged endothelium, particularly within the hepatic sinusoids. This consumption of platelets produces thrombocytopenia. As platelets deposit fibrin strands across damaged microvasculature, red blood cells are mechanically sheared while passing through these partially occluded vessels, resulting in microangiopathic hemolytic anemia (the hallmark schistocytes on peripheral smear, elevated LDH, low haptoglobin, and elevated indirect bilirubin).

Within the liver, fibrin deposition in the sinusoids causes periportal hepatocellular necrosis, which drives the rise in AST and ALT. As necrosis expands, subcapsular hemorrhage may develop, creating hepatic hematomas. In the most catastrophic scenario, a subcapsular hematoma ruptures, leading to hepatic rupture with hemoperitoneum, a life-threatening surgical emergency. This is why RUQ pain in HELLP syndrome should never be dismissed; it reflects hepatic capsular distension from ongoing hemorrhage or necrosis beneath the capsule.

The connection between pathophysiology and symptoms is direct: the malaise and nausea reflect hepatic inflammation (patients often feel "flu-like"), the RUQ pain reflects capsular distension, the thrombocytopenia explains any bleeding tendency or petechiae, and the hemolysis explains jaundice or dark urine when present. An important point is that hypertension and proteinuria may be mild or even absent in up to 15% of HELLP cases. This is a frequent source of diagnostic delay, because clinicians (and exam-takers) anchor too heavily on the blood pressure.

The syndrome most commonly manifests between 28 and 36 weeks of gestation, but roughly 30% of cases present postpartum, typically within 48 hours of delivery. Postpartum HELLP may develop de novo, which is a tested scenario designed to see whether students recognize the disease outside the expected antepartum window.

2. Classification and Clinical Manifestation

The most widely referenced classification system is the Mississippi (Martin) classification, which stratifies HELLP by the degree of thrombocytopenia, the strongest predictor of maternal morbidity.

Mississippi Classification

Class	Platelet Count	LDH	AST or ALT
Class 1 (Severe)	Less than or equal to 50,000/microL	Greater than or equal to 600 IU/L	Greater than or equal to 70 IU/L
Class 2 (Moderate)	50,000 to 100,000/microL	Greater than or equal to 600 IU/L	Greater than or equal to 70 IU/L
Class 3 (Mild)	100,000 to 150,000/microL	Greater than or equal to 600 IU/L	Greater than or equal to 40 IU/L

Tennessee Classification

Category	Criteria
Complete HELLP	All three components present: hemolysis (LDH > 600, abnormal smear), AST > 70, platelets < 100,000
Partial HELLP	Only one or two of the three components are present (e.g., elevated liver enzymes with thrombocytopenia but no hemolysis)

The Tennessee system is clinically useful because partial HELLP can either resolve spontaneously or progress to complete HELLP. Patients with partial HELLP require close surveillance because a proportion will deteriorate. For exam purposes, the Mississippi system is more commonly tested when a question asks about severity grading, while the Tennessee distinction matters when a vignette presents an "incomplete" laboratory picture and asks whether this qualifies as HELLP.

Clinical Manifestation by Organ System

System	Manifestation	Mechanism
Hepatic	RUQ or epigastric pain, nausea, vomiting, elevated transaminases, subcapsular hematoma, hepatic rupture	Fibrin deposition in sinusoids, periportal necrosis, capsular distension
Hematologic	Thrombocytopenia, petechiae, bleeding from IV sites, DIC (in advanced cases)	Platelet consumption at sites of endothelial damage
Hemolytic	Fatigue, jaundice, dark urine, schistocytes, elevated LDH, low haptoglobin	Mechanical shearing of erythrocytes through fibrin-laden microvasculature
Renal	Oliguria, elevated creatinine, proteinuria	Glomerular endotheliosis (shared with preeclampsia)
Neurological	Headache, visual disturbances, seizures (if eclampsia co-occurs)	Cerebral vasospasm and vasogenic edema
Placental	Fetal growth restriction, non-reassuring fetal heart tracing, placental abruption	Uteroplacental insufficiency from spiral artery disease

3. Diagnostic Workup

Test	Role	Key Findings
Peripheral blood smear	Best initial evidence of hemolysis	Schistocytes (fragmented red cells), polychromasia, helmet cells
LDH	Hemolysis and tissue damage marker	> 600 IU/L (some sources use > 2x upper limit of normal)
Haptoglobin	Confirmatory for intravascular hemolysis	Low or undetectable (consumed by binding free hemoglobin)
Indirect bilirubin	Supportive hemolysis marker	Elevated due to RBC destruction
AST and ALT	Liver injury assessment	AST typically > 70 IU/L; ALT also elevated but AST often predominates
Platelet count	Defines the syndrome and its severity	< 100,000/microL for complete HELLP
Coagulation panel (PT, aPTT, fibrinogen, D-dimer)	Screen for DIC	Prolonged PT/aPTT, low fibrinogen (< 300 mg/dL is concerning; < 200 is consistent with DIC), elevated D-dimer
Serum creatinine and BUN	Renal function	Elevated if renal involvement is present
Urinalysis or protein/creatinine ratio	Proteinuria assessment	May or may not be positive
Liver ultrasound	Evaluate for subcapsular hematoma or hepatic rupture	Indicated when RUQ pain is severe, persistent, or worsening; may show perihepatic fluid or subcapsular collection
CT abdomen (with contrast)	Further evaluate hepatic complications	Reserved for suspected hepatic rupture or when ultrasound is equivocal

The diagnosis of HELLP syndrome is made by laboratory criteria, not by any single imaging study. There is no "gold standard" imaging test. The diagnosis requires demonstrating all three components of the triad: hemolysis, elevated liver enzymes, and low platelets. The best initial step when HELLP is suspected is to send a CBC with peripheral smear, LDH, haptoglobin, liver function panel, and coagulation studies simultaneously. Waiting for one test before ordering the next wastes time in a rapidly progressive disease.

The peripheral blood smear deserves emphasis. Finding schistocytes on the smear is what confirms the hemolysis is microangiopathic in nature, distinguishing HELLP from other causes of thrombocytopenia in pregnancy (such as gestational thrombocytopenia or immune thrombocytopenic purpura, where the smear shows large platelets but no fragmented red cells). The smear, combined with elevated LDH and low haptoglobin, establishes the hemolytic component with confidence.

Haptoglobin is the most sensitive marker for intravascular hemolysis. When red cells are destroyed within the vasculature, free hemoglobin is released and binds haptoglobin, depleting it. A very low or undetectable haptoglobin in a pregnant woman with thrombocytopenia and elevated liver enzymes essentially clinches the diagnosis.

Coagulation studies are essential because HELLP can progress to frank disseminated intravascular coagulation (DIC). Fibrinogen is an acute-phase reactant that is normally elevated in pregnancy (typical third-trimester fibrinogen is 400 to 600 mg/dL). Therefore, a fibrinogen level that appears "normal" by non-pregnant standards (e.g., 250 mg/dL) is actually low in the context of pregnancy and should raise concern for consumptive coagulopathy.

Liver imaging is not required for diagnosis but becomes critical when hepatic complications are suspected. If the patient has severe or worsening RUQ pain, shoulder pain (referred from diaphragmatic irritation by a subcapsular hematoma), or hemodynamic instability out of proportion to visible blood loss, an urgent liver ultrasound or CT should be performed to evaluate for subcapsular hematoma or rupture.

4. Management and Treatment

Phase	Intervention	Details
Seizure prophylaxis	Magnesium sulfate	Loading: 4 to 6 g IV over 15 to 20 minutes; Maintenance: 1 to 2 g/hour IV infusion; continue 24 to 48 hours postpartum
Severe hypertension control	IV labetalol or IV hydralazine	Labetalol: 20 mg IV, escalating to 40 mg then 80 mg q10-15 min (max 300 mg). Hydralazine: 5 to 10 mg IV q15-20 min. Target: BP < 160/110
Antenatal corticosteroids (fetal lung maturity)	Betamethasone or dexamethasone	Betamethasone 12 mg IM q24h x 2 doses, or dexamethasone 6 mg IM q12h x 4 doses; indicated if < 34 weeks
Platelet transfusion	For active bleeding or prior to cesarean	Transfuse if platelets < 20,000 (general threshold) or < 50,000 if cesarean delivery is planned or active bleeding is present
Correction of coagulopathy	Fresh frozen plasma, cryoprecipitate, packed RBCs	FFP for prolonged PT/INR; cryoprecipitate if fibrinogen < 200 mg/dL; pRBCs for hemoglobin < 7 g/dL or symptomatic anemia
Definitive treatment	Delivery	Indicated at any gestational age once maternal stabilization is achieved; route determined by obstetric factors
Postpartum monitoring	Serial labs q6-12 hours	Monitor platelet count, LDH, liver enzymes; expect nadir at 24 to 48 hours postpartum with recovery by day 4 to 5

Step 1: Recognize and stabilize. When laboratory studies return the classic triad (hemolysis, elevated liver enzymes, thrombocytopenia), the immediate priorities are seizure prophylaxis with magnesium sulfate and blood pressure control if hypertension is in the severe range. Even if the patient does not currently have preeclampsia-range blood pressures, magnesium sulfate is still given because HELLP carries a high risk of seizure progression.

Step 2: Assess for complications. Before planning delivery, evaluate for DIC (coagulation panel and fibrinogen), hepatic complications (ultrasound if severe RUQ pain), and fetal status (non-stress test or biophysical profile). If DIC is present, correct coagulopathy with FFP and cryoprecipitate before proceeding to delivery.

Step 3: Administer corticosteroids if indicated. If the gestational age is less than 34 weeks, give betamethasone 12 mg IM every 24 hours for two doses for fetal lung maturation. However, unlike mild preeclampsia where expectant management to gain steroid benefit is standard practice, in HELLP syndrome delivery should not be delayed beyond 24 to 48 hours solely for steroid completion if the maternal condition is deteriorating. Maternal stability takes priority over completing the steroid course.

A frequently tested nuance involves the use of dexamethasone at higher doses (10 mg IV every 12 hours) to improve the maternal platelet count and liver function in HELLP. This practice (sometimes called "maternal-benefit" steroids) has been studied and remains controversial. Current evidence does not definitively support that high-dose dexamethasone alters meaningful maternal outcomes (such as preventing hepatic rupture or DIC), and major guidelines do not universally recommend it for maternal indications. On an exam, the safest answer regarding steroids in HELLP is to give them for fetal lung maturity if < 34 weeks, and to proceed with delivery rather than delay for a maternal steroid response.

Step 4: Plan delivery. Unlike uncomplicated preeclampsia with mild features where expectant management to 37 weeks may be reasonable, HELLP syndrome at any gestational age is an indication for delivery after maternal stabilization, typically within 24 to 48 hours of diagnosis. The route of delivery depends on standard obstetric factors. Vaginal delivery is preferred when the cervix is favorable and there are no contraindications. Cesarean section is performed for the usual obstetric indications (malpresentation, non-reassuring fetal heart tracing, failed induction). If the platelet count is below 50,000, general anesthesia is required because neuraxial anesthesia (spinal or epidural) is contraindicated due to the risk of epidural or spinal hematoma.

Step 5: Postpartum monitoring. HELLP does not resolve immediately after delivery. The platelet count typically reaches its nadir at 24 to 48 hours postpartum, then begins recovering. Liver enzymes and LDH follow a similar trajectory. Serial labs every 6 to 12 hours should be drawn to confirm the trend is improving. Most patients show meaningful recovery by postpartum day 4 to 5. If the labs continue to deteriorate beyond 72 hours postpartum, consider alternative diagnoses such as thrombotic thrombocytopenic purpura (TTP) or hemolytic uremic syndrome (HUS), which do not improve with delivery.

Contraindications and cautions: Magnesium sulfate must be dose-reduced and monitored closely in patients with renal impairment (reduce maintenance to 1 g/hour, monitor serum levels directly). Calcium gluconate (10 mL of 10% solution IV) should be at the bedside as the antidote for magnesium toxicity. Labetalol is contraindicated in patients with asthma or severe bradycardia; in these cases, use hydralazine or nifedipine. Avoid ACE inhibitors and ARBs in pregnancy at all times.

5. Differential Diagnosis and Distractors

Differential Diagnosis	Why It Looks Similar	Key Discriminator
Acute fatty liver of pregnancy (AFLP)	Third-trimester woman with nausea, vomiting, RUQ pain, liver dysfunction, and possible coagulopathy	AFLP features hypoglycemia (a hallmark finding absent in HELLP), markedly elevated bilirubin, prolonged PT from hepatic synthetic failure, and often low fibrinogen without overt hemolysis; peripheral smear lacks schistocytes; liver biopsy (rarely needed) shows microvesicular steatosis
Thrombotic thrombocytopenic purpura (TTP)	Microangiopathic hemolytic anemia with thrombocytopenia, schistocytes, and neurological symptoms in pregnancy	TTP presents with the classic pentad (hemolytic anemia, thrombocytopenia, neurological changes, renal dysfunction, fever); ADAMTS13 activity is severely deficient (< 10%); liver enzymes are usually normal or only mildly elevated; does not improve after delivery
Hemolytic uremic syndrome (HUS)	Microangiopathic hemolytic anemia and thrombocytopenia; can occur postpartum	HUS is dominated by renal failure (more severe than in HELLP), often with bloody diarrhea if triggered by Shiga toxin; liver enzymes are not a prominent feature; does not resolve with delivery
Immune thrombocytopenic purpura (ITP)	Isolated thrombocytopenia in pregnancy	ITP shows isolated low platelets without hemolysis (no schistocytes, normal LDH, normal haptoglobin) and without elevated liver enzymes; the peripheral smear shows large platelets but no fragmented cells
Gestational thrombocytopenia	Low platelets discovered incidentally in the third trimester	Platelet count is usually mildly reduced (100,000 to 150,000), never below 70,000; no hemolysis, no liver enzyme elevation; completely benign and resolves postpartum
Viral hepatitis	Nausea, vomiting, RUQ pain, elevated liver enzymes	Viral serologies (hepatitis A IgM, hepatitis B surface antigen, hepatitis C antibody) are positive; no thrombocytopenia or hemolysis; clinical context includes risk factors for viral exposure
Preeclampsia with severe features (without HELLP)	Hypertension, proteinuria, elevated liver enzymes in the third trimester	Severe preeclampsia may have elevated liver enzymes alone, but lacks the hemolytic component (no schistocytes, normal LDH and haptoglobin) and platelet count may be normal or only borderline low
Cholecystitis / Cholelithiasis	RUQ pain, nausea, vomiting in pregnancy	Murphy sign positive; RUQ ultrasound shows gallstones or gallbladder wall thickening; labs show elevated alkaline phosphatase and possibly elevated direct bilirubin, but platelet count and LDH are normal; no hemolysis

6. Traps and High-Yield Pearls

The most frequent mistake students make with HELLP syndrome is failing to consider it when hypertension is absent or mild. Approximately 15% of HELLP patients present without hypertension, and up to 13% may have no proteinuria. This means that a third-trimester woman with nausea, epigastric pain, and "normal" blood pressure can still have HELLP. The exam exploits this by presenting a vignette that looks like gastritis, reflux, or a viral syndrome, then burying the diagnostic clue in the laboratory results (thrombocytopenia, elevated LDH, elevated transaminases). Always check the labs carefully when a pregnant woman in the third trimester presents with upper abdominal complaints, even if the blood pressure appears reassuring.

A second trap involves confusing HELLP with AFLP. Both present in the third trimester with liver dysfunction and coagulopathy. The discriminator tested most often is hypoglycemia: AFLP causes hepatic synthetic failure severe enough to impair gluconeogenesis, producing a characteristically low serum glucose. HELLP does not. If the vignette mentions low blood glucose alongside liver dysfunction in late pregnancy, think AFLP, not HELLP.

A third trap targets the distinction between HELLP and TTP/HUS. All three feature microangiopathic hemolytic anemia and thrombocytopenia. The tested discriminator is the response to delivery and the dominant organ involvement. HELLP improves after delivery (labs begin recovering within 48 to 72 hours). TTP and HUS do not improve with delivery and require their own treatments (plasma exchange for TTP, supportive care or eculizumab for atypical HUS). If a postpartum patient's labs continue to worsen after delivery, the question is testing whether you can pivot away from HELLP toward TTP or HUS.

A fourth trap involves platelet transfusion thresholds. Students often transfuse reflexively when they see a low number. In HELLP, the threshold for transfusion is < 20,000 for a non-bleeding patient, or < 50,000 if cesarean delivery is planned or the patient is actively bleeding. Transfusing at higher thresholds wastes blood products and does not improve outcomes, because the underlying consumptive process rapidly destroys transfused platelets.

Finally, remember the neuraxial anesthesia cutoff: epidural and spinal anesthesia are contraindicated when platelets are below 50,000 to 75,000 (depending on the guideline referenced; on an exam, < 50,000 is the most commonly cited threshold). A vignette describing a HELLP patient going to cesarean with a platelet count of 40,000 is testing whether you choose general anesthesia over regional.

The core competency being tested in HELLP questions is pattern recognition in an atypical presentation (RUQ pain with flu-like symptoms rather than classic hypertensive features), the ability to read a lab panel and assemble the triad, and correct sequencing of stabilization followed by delivery as the definitive treatment.

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