HELLP syndrome
Background
- HELLP = Hemolysis, Elevated LFTs, Low Platelets
- Clinical variant of preeclampsia
- Any patient >20wk gestation or <4wk postpartum complaining of abdominal pain should receive HELLP workup
- When occurring postpartum, typically presents within 48hr of delivery
- 80% of patients have no evidence of preeclampsia before delivery
Clinical Features
- RUQ or epigastric pain (40-90%)
- Proteinuria (86-100%)
- Hypertension (82-88%)
Complications
- Disseminated intravascular coagulation
- Acute kidney injury
- Subcapsular liver hematoma
- Hemorrhage
Differential Diagnosis
3rd Trimester/Postpartum Emergencies
- Acute fatty liver of pregnancy
- Amniotic fluid embolus
- Chorioamnionitis
- Eclampsia
- HELLP syndrome
- Mastitis
- Peripartum cardiomyopathy
- Postpartum endometritis (postpartum PID)
- Postpartum headache
- Postpartum hemorrhage
- Preeclampsia
- Resuscitative hysterotomy
- Retained products of conception
- Septic abortion
- Uterine rupture
Microangiopathic Hemolytic Anemia (MAHA)
- Disseminated Intravascular Coagulation (DIC)
- Thrombotic Thrombocytopenic Purpura (TTP)
- Hemolytic Uremic Syndrome (HUS)
- HELLP syndrome
- Heparin-Induced Thrombocytopenia (HIT)
- Hereditary spherocytosis
- Paroxysmal nocturnal hemoglobinuria (PNH)
- Malignant hypertension
- Scleroderma
- Antiphospholipid Syndrome (APS)
- Other medical causes: malignancy, renal allograft rejection, vasculitides like polyarteritis nodosa and Wegener's granulomatosis
- Drugs: chemotherapy; Clopidogrel (Plavix) associated with TTP
- Nonvascular causes: prosthetic valve (more common with mechanical, more common at aortic valve), LVAD, TIPS, coil embolization, patched AV shunt, AVM
Jaundice
Indirect Hyperbilirubinemia
- Hemolytic
- G6PD
- Drug related
- Autoimmune hemolytic anemia
- Hematoma resorption
- Ineffective erythropoiesis
- Gilbert's
Direct (Conjugated) Hyperbilirubinemia
- Choledocholithiasis
- Cholecystitis
- Ascending cholangitis
- AIDS cholangiopathy
- Stricture
- Neoplasm
- Pancreatic head
- Gallbladder
- Primary liver (e.g. hepatocellular carcinoma)
- Metastatic
- Obstructing AAA
Hepatocellular damage
Patient will have severely elevated AST/ALT with often normal Alkaline Phosphatase
- Viral hepatitis
- Fulminant hepatic failure
- alcoholic hepatitis
- Ischemic hepatitis
- Toxins
- Isoniazid
- Phenytoin
- acetaminophen
- Ritonavir
- Halothane
- Sulfonamide
- Autoimmune hepatitis
- Primary biliary cirrhosis
- HELLP Syndrome
- Congestive Hepatopathy
Pregnancy Related
Transplant Related
Pediatric Related
- Inborn error of metabolism
- Neonatal jaundice (physiologic)
Additional Differential Diagnosis
- Reye syndrome
- TPN
- Heatstroke
- Budd-Chiari (with acute ascites)
- Wilson's disease
- Sarcoidosis
- Amyloidosis
Masqueraders
Only bilirubin stains the sclera
- Carotenemia
- Quinacrine ingestion
- Dinitrophenol, teryl (explosive chemicals)
Evaluation
Evaluation is targeted at assessing the individual components of the syndrome. Labs to be drawn should include a:
- CBC
- May see a microangiopathic hemolytic anemia (Schistocytes on microscopy)
- Platelet count <150 × 109 per L (150 per mm3)
- Chemistry
- Normal or elevated BUN/Cr
- Coagulation panel (fibrinogen can be decreased and PTT elevated)
- Patients can progress towards DIC
- LFTs
- AST levels > 150 IU/L and ALT levels > 100 IU/L (also often associated with bilirubin > 1.2mg/dL
- LDH elevation (often > 600 IU/L)
- UA (proteinuria)
- CT or ultrasound can be considered if there is pain requiring a greater workup.
- Depending on degree of thrombocytopenia are at risk for developing intraabdominal bleeding such as a hepatic hematoma
Management
- Similar to that of severe preeclampsia or eclampsia
- Delivery (only definitive treatment)
- Magnesium sulfate
- BP control
- Coagulopathy correction
- Transfuse platelets if < 20,000 or active, uncontrolled bleeding
Disposition
- Admit