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HELLP Syndrome Workup

  • Author: Huma Khan, MD; Chief Editor: Ronald M Ramus, MD  more...
 
Updated: Dec 30, 2015
 

Imaging Studies

Although rare, large-vessel vasculopathy could result in hepatic infarction or subcapsular hematomas. If suspected (usually correlated with worsening hepatic function test results), CT scanning or MRI should be obtained. Hepatic ultrasonography may reveal increased echogenicity in irregular, well-demarcated areas of the liver.[20, 2]

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Histologic Findings

Hepatic endothelial disruption and subsequent platelet activation, aggregation, and consumption lead to distal ischemia and hepatocyte death, which can be segmental or apparent diffusely throughout the liver.[32] Since HELLP tends to involve smaller terminal arterioles, characteristic histiologic features are periportal or focal parenchymal necrosis with hyaline deposits of fibrinlike material in the sinusoids.[33, 34, 35] If larger-vessel vasculopathy occurs, hepatic infarction or subcapsular hematomas may result, both of which would require imaging studies such as MRI or CT scanning.[36]

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Staging

Two common classifications used to predict maternal morbidity and mortality were described in and are known as the Mississippi and the Tennessee classifications.

The two methods of classification, while useful, should not be regarded as a hard and fast rule. Partial HELLP syndrome, as per the Tennessee classification, can progress to the complete form. In addition, increased eclampsia and higher perinatal morbidity and mortality have been demonstrated in patients with HELLP syndrome,[37] while those with Mississippi class 3 disease have been shown to exhibit hepatic rupture.[38, 39]

Mississippi classification

The Mississippi classification divides HELLP syndrome into 3 classes based on platelet count, AST or ALT levels, and LDH levels.

Class 1, which has an approximately 13% incidence of bleeding, is associated with the highest maternal morbidity and mortality rates and longest recovery time. Patients in this class have a platelet count less than 50,000/µL, liver dysfunction with AST or ALT levels greater than 70 IU/L, and hemolysis as evidenced by an LDH level greater than 600 IU/L.

Class 2 includes platelet counts from 50,000-100,000/µL with AST, ALT, and LDH levels similar to those in class 1. Class 2 has an 8% incidence of bleeding.

The mild form of HELLP, class 3, has a platelet count from 100,000-150,000/µL, AST and ALT greater than 40 IU/L, and LDH greater than 600 IU/L, with no increased risk of bleeding.

The more severe the class, the longer the recovery time postpartum.[23, 38]

Table 2: Mississippi Classification of HELLP Syndrome (Open Table in a new window)

  Class 1 (Severe) Class 2 (Moderate) Class 3 (Mild)
Platelets ≤50,000/µL 50,000-100,000/µL 100,000-150,000/µL
AST or ALT ≥70 IU/L ≥70 IU/L ≥40 IU/L
LDH ≥600 IU/L ≥600 IU/L ≥600 IU/L
Incidence of bleeding 13% 8% No increased risk

 

Tennessee classification

The Tennessee classification describes HELLP as either complete or partial.

Complete HELLP is defined as hemolysis with an abnormal peripheral smear finding and an LDH level greater than 600 IU/L or bilirubin level greater than 1.2 mg/dL. Patients with complete HELLP have platelet counts less than 100,000/µL and AST levels over 70 IU/L.

Partial HELLP describes severe preeclampsia plus some features of HELLP. These features are further defined as LP, or low platelet syndrome (slightly thrombocytopenic but no hemolysis or liver dysfunction); EL, or elevated liver enzyme syndrome (mildly elevated liver enzymes but no hemolysis or thrombocytopenia); HEL syndrome (hemolysis, elevated liver enzyme levels, but no thrombocytopenia); and ELLP syndrome (elevated liver enzyme levels and low platelet counts, no hemolysis).[38]

Complete HELLP syndrome is characterized by the following:

  • Platelet count of 100,000/μL or less
  • AST or ALT levels of 70 IU/L or more
  • LDH (or bilirubin) (with hemolysis as evidenced on abnormal peripheral smear) levels of 600 IU/L (≥0.2 mg/dL) or more

Partial HELLP syndrome is characterized by severe preeclampsia plus one of the following:

  • ELLP: Elevated liver enzyme levels, thrombocytopenia, no hemolysis
  • EL: Mildly elevated liver enzyme levels, no thrombocytopenia, no hemolysis
  • LP: Thrombocytopenia, no hemolysis, normal liver enzyme levels
  • HEL: Hemolysis, liver dysfunction, no thrombocytopenia
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Laboratory Studies

Laboratory evaluation should include the following:

  • Type and screen
  • Complete blood cell (CBC) count: Thrombocytopenia, anemia with possible reticulocytosis
  • Coagulation studies: Normal prothrombin time, 50% may have prolonged activated partial thromboplastin time
  • Peripheral smear: Schistocytes, helmet cells, and burr cells secondary to microangiopathic hemolytic anemia
  • Serum aspartate aminotransferase/alanine aminotransferase (AST/ALT) levels: Elevated secondary to liver dysfunction
  • Lactate dehydrogenase (LDH) level: Elevated secondary to liver dysfunction or hemolysis
  • Complete metabolic panel (CMP): Elevated blood urea nitrogen (BUN)/creatinine with acute renal failure
  • Bilirubin level: Increased secondary to hemolysis
  • Haptoglobin level: Decreased secondary to hemolysis
  • Fibrinogen levels: Low secondary to increased coagulation
  • D-dimer: Increased due to fibrinolysis/DIC

Laboratory abnormalities apparent in HELLP syndrome and the recovery time postpartum required for normalization of these findings are summarized in Table 1.[22, 2]

Table 1: Laboratory Findings in HELLP Syndrome (Open Table in a new window)

Laboratory Test Possible Result Cause Recovery to Baseline (in Number of Hours or Days Postpartum)
Haptoglobin Hemolysis 24-30 hours
LDH Hemolysis or liver dysfunction 3-5 days
AST or ALT Liver dysfunction 3-5 days
Bilirubin Hemolysis -
Platelets (CBC) Consumption 6-11 days
Hemoglobin/Hematocrit (CBC) Hemolysis -
PT Normal    
PTT Liver dysfunction -
D-dimer Increased coagulation and secondary fibrinolysis -
Fibrinogen -
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Contributor Information and Disclosures
Author

Huma Khan, MD Resident Physician, Department of Emergency Medicine, Northshore-Long Island Jewish Medical Center

Disclosure: Nothing to disclose.

Coauthor(s)

Natalie B Meirowitz, MD Assistant Professor of Obstetrics and Gynecology, Albert Einstein College of Medicine; Chief of Maternal-Fetal Medicine, Director, Center for Maternal-Fetal Health, Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Northshore-Long Island Jewish Medical Center

Natalie B Meirowitz, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, Society for Maternal-Fetal Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Ronald M Ramus, MD Professor of Obstetrics and Gynecology, Director, Division of Maternal-Fetal Medicine, Virginia Commonwealth University School of Medicine

Ronald M Ramus, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, American Institute of Ultrasound in Medicine, Medical Society of Virginia, Society for Maternal-Fetal Medicine

Disclosure: Nothing to disclose.

Additional Contributors

Jordan G Pritzker, MD, MBA, FACOG Adjunct Professor of Obstetrics/Gynecology, Hofstra North Shore-LIJ School of Medicine at Hofstra University; Attending Physician, Department of Obstetrics and Gynecology, Long Island Jewish Medical Center; Medical Director, Aetna, Inc; Private Practice in Gynecology

Disclosure: Nothing to disclose.

References
  1. Rahman TM, Wendon J. Severe hepatic dysfunction in pregnancy. Q J Med. 2002. 95:343:[Medline].

  2. Lichtman, M, Kipps T, Seligsohn U, Kaushansky K, Prchal J. Hemolytic Anemia resulting from physical Injury to Red Cells. Williams Hematology, Eighth Edition. 8. McGraw-Hill Companies; 2010. Chapter 50.

  3. Weinstein L. Syndrome of hemolysis, elevated liver enzymes, and low platelet count: A severe consequence of hypertension in pregnancy. Am J Obstet Gynecol. 1982. 142:159. [Medline].

  4. Lichtman, M, Kipps T, Seligsohn U, Kaushansky K, Prchal J. Thrombocytopenia. Williams Hematology, Eighth Edition. 8. McGraw-Hill Companies; 2010. Chapter 119.

  5. Knerr I, Beinder E, Rascher W. Syncytin, a novel human endogenous retroviral gene in human placenta: Evidence for its dysregulation in preeclampsia and HELLP syndrome. Am J Obstet Gynecol. 2002. 186:210. [Medline].

  6. Levine RJ, Maynard SE, Qian C, et al. Circulating angiogenic factors and the risk of preeclampsia. N Engl J Med. 2004. 350:672. [Medline].

  7. Mutter WP, Karumanchi SA. Molecular mechanisms of preeclampsia. Microvasc Res. 2008. 75:1. [Medline].

  8. Widmer M, Villar J, Beniani A, et al. Mapping the theories of preeclampsia and the role of angiogenic factors: A systematic review. Obstet Gynecol 109:168, 2007. Obstet Gynecol. 2007. 109:168. [Medline].

  9. Semenovskaya Z, Erogul M. Pregnancy, Preeclampsia. Medscape Reference. May 2010. [Full Text].

  10. Zhou Y, McMaster M, Woo K, et al. Vascular endothelial growth factor ligands and receptors that regulate human cytotrophoblast survival are dysregulated in severe preeclampsia and hemolysis, elevated liver enzymes, and low platelets syndrome. Am J Pathol. 2002. 160:1405-23. [Medline].

  11. Nelson J, Lewis B, Walters B. The HELLP syndrome associated with fetal medium chain acyl-CoA dehydrogenase deficiency. Journal of Inherited Metabolic Diseases. 2000. 23:518-519.

  12. Ibdah JA, Bennet MJ, Rinaldo P, Zhao Y, Gibson B, Sims HF, et al. A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women. New England Journal of Medicine. 1999. 340:1723-1731.

  13. Strand S, Strand D et al. Placenta-derived CD59 ligand causes liver damage in hemolysis, elevated liver enzymes and low platelet count syndrome. Gastroenterology. 2004. 126:849-858.

  14. Fang C, Richards A et al. Advances in understanding of pathogenesis of aHUS and HELLP. BJH British Journal of Haematology. 2008. 143:336-348.

  15. Ohara Padden, M. HELLP Syndrome: Recognition and Perinatal Management. The Academy of Family Physicians. Sept 1, 1999. [Full Text].

  16. Martin JN Jr, Magann EF, Blake PG. Analysis of 454 pregnancies with severe preeclampsia/eclampsia/HELLP syndrome using the 3-class system of classification. Am J Obstret Gynecol 1993. 1993. 168:386.

  17. Martin JN Jr, Magann EF. HELLP syndrome current principles and recommended practice. Curr Obstet Med. 1996. 4:129-75.

  18. Sibai BM, Ramamdan MK, Usta I, Salama M, Mercer BM, Friedman SA. Maternal morbidity and mortality in 442 pregnancies with HELLP syndrome. Am J Obstet Gynecol. 1993. 169:1000-6.

  19. Ukomadu C, Greenberger N, Blumberg R, Burakoff R. Hepatic Complications of Pregnancy. Current Diagnosis and Treatment: Gastroenterology, Hepatology and Endoscopy. McGraw Hills and Company; 2009. Chapter 8.

  20. O’Brien JM, Barton JR. Controversies with the diagnosis and management of HELLP syndrome. Clinical Obstetrics and Gynecology. June 2005. 48:2:460-477.

  21. Sullivan CA, Magann EF, Perry KG Jr, et al. The recurrence risk of the syndrome of hemolysis, elevated liver enzymes, and low platelets: Subsequent pregnancy outcome and long term prognosis. Am J Obstet Gynecol. 1995. 172:125.

  22. Barton JR, Sibai BM. Diagnosis and management of hemolysis, elevated liver enzymes, and low platelets syndrome. Clin Perinatol. 2004. 31:807-33. [Medline].

  23. Magann EF, Martin JN Jr. Twelve steps to optimal management of HELLP syndrome. Clin Obstet Gynecol. 1999. 42:532. [Medline].

  24. Isler CM, Rinehart CK, Terrone DA, Martin RW, Magann EF, Martin JN Jr. Maternal mortality associated with HELLP syndrome. Am J Obstet Gynecol. 1999. 181:924-8.

  25. Rath W, Faridi A, Dudenhausen JW. HELLP syndrome. J Perinat Med. 2000. 28:249. [Medline].

  26. Harms K, Rath W, Herting E, Kuhn W. Maternal hemolysis, elevated liver enzymes, low platelet count, and neonatal outcome. Am J Perinatol. 1995. 12:1:[Medline].

  27. Sibai BM. Diagnosis, controversies and management of the syndrome of hemolysis, elevated liver enzymes and low platelet count. Obstet and Gynecol. 2004. 103:981-91.

  28. Vigil-De Gracia P. Pregnancy complicated by pre-eclampsia-eclampsia with HELLP syndrome. Int J Gynecol Obstet. 2001. 72:17-23.

  29. Martin JN Jr. Rinehart K, May WL, Magann EF, Terrone DA, Blake PG. The spectrum of severe preeclampsia: comparative analysis by HELLP syndrome classification. Am J Obstet Gynecol. 1999. 180:1373-84.

  30. Sibai B. HELLP Syndrome. UptoDate. 2010. [Full Text].

  31. Weinstein L. Preeclampsia/eclampsia with hemolysis, elevated liver enzymes and thrombocytopenia. Obstet Gynecol. 1985. 66:657- 60.

  32. Roberts JM. Cooper DW. Pathogenesis and genetics of pre-eclampsia. Lancet. 2001. 357:53-56.

  33. Aarnoudse JG, Houthhoff HF et al. A syndrome of liver damage and intravascular coagulation in the last trimester of normotensive pregnancy. A clinical and histopathological study. BR J Obstet Gynaecol. 1986. 93:145-155.

  34. Arias F, Mancilla-Jimenez R. Hepatic fibrinogen deposits in pre-eclampsia. Immunofluorescent evidence. N Engl J Med. 1976. 295:578-582.

  35. Barton JR, Riely CA, Adamel TA, et al. Hepatic histopathologic condition does not correlate with laboratory abnormalities in HELLP syndrome (hemolysis, elevated liver enzymes and low platelet count). Am J Obstet Gynecol. 1992. 167:1538-1543.

  36. Barton JR, Sibai BM. Hepatic imaging in HELLP syndrome. Am J Obstet Gynecol. 1996. 174:1820-1827.

  37. Martin JN Jr., Magann EF, Isler CM. HELLP Syndrome: the scope of disease and treatment. HELLP Syndrome: the scope of disease and treatment. Belfort MA. Thornton S, Saade GR. Hypertension in Pregnancy. Oxford:Marcel Dekker; 2003. Chapter 7 p 141-88.

  38. Martin JN Jr. Rose C, Briery C. Understanding and managing HELLP syndrome: The integral role of aggressive glucocorticoids for mother and child. Am J of Obst & Gyn. 2006. 195:914-34.

  39. Van Runnard Heimel PH, Juisjes AJM, Franx A, Koopman C, Bots ML, Bruinse HW. A randomized placebo-controlled trial of prolonged administration to patients with HELLP syndrome remote from term: maternal and neonatal complications. Am J Obstet Gynecol. 2004. 191:S41.

  40. Isler CM, Barrilleaux PS, Magann EF et al. A prospective randomized trial comparing the efficacy of dexamethasone and betamethasone for the treatment of antepartum HELLP syndrome. Am J Obstet Gynecol. 2001. 184:1332-39.

  41. O’Brien JM, Milligan DA, Barton JR. Impact of high-dose corticosteroid therapy for patients with HELLP (hemolysis, elevated liver enzymes and low platelet count) syndrome. Am J Obstet Gynecol. 2000. 183:921-4.

  42. Briggs R, Chari RS, Mercer B, Sibai BM. Postoperative incision complications after cesareansection in patients with antepartum syndrome of hemolysis, elevated liver enzymes, and lowplatelets (HELLP): does delayed primary closure make a difference?. Am J Obstet Gynecol. 1996. 175:183-6.

  43. Allen AM, Kim WR, Larson JJ, et al. The epidemiology of liver diseases unique to pregnancy in a US community: a population-based study. Clin Gastroenterol Hepatol. 2015 Aug 21. [Medline].

  44. Morisawa H, Makino S, Takahashi H, Sorita M, Matsubara S. Retinal detachment in hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome: Color vision abnormality as the first and predominant manifestation. J Obstet Gynaecol Res. 2015 Nov. 41(11):1835-8. [Medline].

 
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Overview of the Management of HELLP Syndrome
Table 2: Mississippi Classification of HELLP Syndrome
  Class 1 (Severe) Class 2 (Moderate) Class 3 (Mild)
Platelets ≤50,000/µL 50,000-100,000/µL 100,000-150,000/µL
AST or ALT ≥70 IU/L ≥70 IU/L ≥40 IU/L
LDH ≥600 IU/L ≥600 IU/L ≥600 IU/L
Incidence of bleeding 13% 8% No increased risk
Table 1: Laboratory Findings in HELLP Syndrome
Laboratory Test Possible Result Cause Recovery to Baseline (in Number of Hours or Days Postpartum)
Haptoglobin Hemolysis 24-30 hours
LDH Hemolysis or liver dysfunction 3-5 days
AST or ALT Liver dysfunction 3-5 days
Bilirubin Hemolysis -
Platelets (CBC) Consumption 6-11 days
Hemoglobin/Hematocrit (CBC) Hemolysis -
PT Normal    
PTT Liver dysfunction -
D-dimer Increased coagulation and secondary fibrinolysis -
Fibrinogen -
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