Guideline sources The following summarized guidelines for the evaluation and management of HELLP syndrome are prepared by our editorial team based on guidelines from the American Association for the Study of Liver Diseases (AASLD 2021), the European Association for the Study of the Liver (EASL 2017), the American College of Gastroenterology (ACG 2016), the Italian Association for the Study of the Liver (AISF 2016), and the Society of Obstetricians and Gynaecologists of Canada (SOGC 2014). 1 2 3 4 5 Guidelines 1. Screening and diagnosis Indications for monitoring: obtain close monitoring for the development of eclampsia and HELLP syndrome in patients with preeclampsia. E 2. Diagnostic investigations Initial evaluation: https://web.pathway.md/diseases/recKa9ufB6qegaEBp 1/3 6/24/23, 12:31 PM HELLP syndrome Pathway As per AASLD 2021 guidelines, obtain abdominal imaging to rule out hepatic hemorrhage, infarct, or rupture in patients with suspected HELLP syndrome. E As per ACG 2016 guidelines, obtain standard workup in a pregnant patient presenting with abnormal liver tests as with any non-pregnant patient. B 3. Medical management Corticosteroids: As per AISF 2016 guidelines, administer corticosteroids to promote fetal lung maturity in patients with HELLP syndrome between 24 and 34 weeks of gestation. Consider planning delivery 24 hours after corticosteroids administration. A As per SOGC 2014 guidelines, do not administer corticosteroids for the treatment of HELLP syndrome. D 4. Therapeutic procedures Platelet transfusion: As per ACG 2016 guidelines, consider administering platelet transfusion to 40,000-50,000 cells/ L before delivery, especially if C-section is likely. C As per SOGC 2014 guidelines, administer platelet transfusion in patients with HELLP syndrome with a platelet count of < 20 109/L, regardless of mode of delivery. B Show 3 more Delivery: As per AASLD 2021 guidelines, perform expeditious deliver after maternal stabilization in patients with suspected HELLP syndrome or eclampsia. E As per ACG 2016 guidelines, perform prompt delivery for the management of HELLP syndrome, especially after 34 weeks gestation. B As per AISF 2016 guidelines, perform delivery of the fetus as first-line treatment of HELLP syndrome before 24 or after 32 weeks of gestation and in presence of maternal-fetal complications. B Plasmapheresis: do not perform plasma exchange or plasmapheresis for HELLP syndrome, particularly within the first 4 days postpartum. D 5. Specific circumstances Patients with acute liver failure: As per AASLD 2021 guidelines, transfer patients with HELLP syndrome complicated by hepatic rupture or ALF to a transplant center for evaluation. E https://web.pathway.md/diseases/recKa9ufB6qegaEBp 2/3 6/24/23, 12:31 PM HELLP syndrome Pathway As per EASL 2017 guidelines, perform prompt delivery of the fetus as the treatment of choice in patients with HELLP syndrome and acute fatty liver of pregnancy, especially in case of elevated lactate levels and hepatic encephalopathy. Offer screening for putative fatty acid defects. B 6. Follow-up and surveillance Breastfeeding: recognize that breastfeeding is not contraindicated in patients with HELLP syndrome on antihypertensive therapy, including nifedipine, labetalol, atenolol, methyldopa, captopril, and enalapril. B References 1. Magee LA, Pels A, Helewa M et al. Diagnosis, evaluation, and management of the hypertensive disorders of pregnancy: executive summary. J Obstet Gynaecol Can. 2014 May;36 5 416 41. Open 2. Tram T Tran, Joseph Ahn, Nancy S Reau. ACG Clinical Guideline: Liver Disease and Pregnancy. Am J Gastroenterol. 2016 Feb;111 2 176 94; quiz 196. Open 3. Monika Sarkar, Carla W Brady, Jaquelyn Fleckenstein et al. Reproductive Health and Liver Disease: Practice Guidance by the American Association for the Study of Liver Diseases. Hepatology. 2021 Jan;73 1 318 365. Open 4. Italian Association for the Study of the Liver AISF , Italian Association for the Study of the Liver AISF. AISF position paper on liver disease and pregnancy. Dig Liver Dis. 2016 Feb;48 2 120 37. Open 5. Wendon, J, Cordoba J et al. EASL Clinical Practical Guidelines on the management of acute (fulminant) liver failure. J Hepatol. 2017 May;66 5 1047 1081. Open 6. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open 7. Laura A Magee, Graeme N Smith, Christine Bloch et al. Guideline No. 426 Hypertensive Disorders of Pregnancy: Diagnosis, Prediction, Prevention, and Management. J Obstet Gynaecol Can. 2022 May;44 5 547 571.e1. Open https://web.pathway.md/diseases/recKa9ufB6qegaEBp 3/3