The Physician's Guide to Laboratory Test Selection and Interpretation

HELLP Syndrome
Diagnosis
Indications for Testing Pregnant patient presenting with clinical picture of preeclampsia, thrombocytopenia, or acute liver failure Criteria for Diagnosis Patients lacking all 3 of the criteria for HELLP (hemolysis, elevated liver enzymes, and low platelets) can be deemed partial HELLP Laboratory Testing CBC anemia, thrombocytopenia (<100,000/mm3 to meet criteria) Peripheral smear helmet cells, burr cells, schistocytes (all signs of microangiopathic hemolysis) Liver function tests elevated Aspartate aminotransferase (AST) 2 times the upper reference limit (to meet criteria) Lactate dehydrogenase (LD) markedly elevated (600 U/L to meet criteria) LD criteria should be based on the upper limits of normal values in the lab where specimen is processed Differential Diagnosis Thrombocytopenia Thrombotic microangiopathies Thrombotic thrombocytopenic purpura (TTP) Hemolytic uremic syndrome (HUS) Disseminated intravascular coagulation Idiopathic thrombocytopenic purpura Systemic lupus erythematosus Antiphospholipid syndrome Sepsis Hemolysis TTP HUS DIC Elevated liver enzymes TTP HUS DIC Acute fatty liver of pregnancy Acute hepatitis Cholangitis Cholecystitis Acute pancreatitis

Clinical Background

HELLP syndrome refers to the constellation of Hemolysis, Elevated Liver function tests, and Low Platelet count seen in pregnant women and sometimes considered to be a severe form of eclampsia. Epidemiology Incidence 4-5/1,000 pregnancies

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The Physician's Guide to Laboratory Test Selection and Interpretation

5-10% of pregnancies with preeclampsia 30-50% of pregnancies with eclampsia Age childbearing years Sex exclusively in pregnant or postpartum females Risk Factors Caucasian race Multiparity Age >34 years Presence of preeclampsia or eclampsia Fetus affected with fatty acid oxidation defect ie, defects such as long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency Pathophysiology Placenta in preeclampsia is poorly perfused, which releases factors that create endothelial dysfunction Endothelial dysfunction causes platelet aggregation and altered ratio of thromboxane to prostacyclin Thrombin-induced activation of the coagulation cascades leads to hemolytic anemia and multiorgan microvascular injury Clinical Presentation Usually occurs during pregnancy 2/3 of patients are diagnosed antepartum Typically does not present prior to 3rd trimester Postpartum presentation usually within first week Symptoms Nonspecific malaise, fatigue Gastrointestinal right upper quadrant pain, nausea, emesis Central nervous system headache, confusion Cardiovascular hypertension Complications Maternal Subcapsular hematomas and liver rupture Abruptio placentae Disseminated intravascular coagulation (DIC) Severe postpartum bleeding Stroke, cerebral hemorrhage Renal failure Increased risk of HELLP in subsequent pregnancies Fetal/perinatal Prematurity Placental insufficiency Intrauterine growth restriction Neonatal intraventricular hemorrhage Treatment Consider fetus delivery Medical emergency with maternal mortality of 1-2% and fetal mortality of 10-30% 34 weeks immediate delivery if maternal status not rapidly improving

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The Physician's Guide to Laboratory Test Selection and Interpretation

<34 weeks but 27 weeks delivery within 48 hours after medical stabilization <27 weeks conservative management based on maternal health Conservative treatment is contraindicated in women with DIC Glucocorticosteroid therapy Nonrandomized studies suggest therapy may improve outcomes Maternal dosing high dose versus repeated dose Fetal dosing based on standard treatment to promote fetal lung maturity

Lab Tests

Indications for Laboratory Testing Tests generally appear in the order most useful for common clinical situations. For test-specific information, refer to the test number in the ARUP Laboratory Test Directory on the ARUP Web site at www.aruplab.com. Test Name and Number Recommended Use Limitations Follow Up

CBC with Platelet Count Use with hepatic function panel and and Automated Differential LD in the diagnosis of HELLP 0040003 Method: Automated Cell Count/Differential Hepatic Function Panel 0020416 Method: Quantitative Enzymatic/Quantitative Spectrophotometry Lactate Dehydrogenase, Serum or Plasma 0020006 Method: Quantitative Enzymatic General References Bacq Y. Liver diseases unique to pregnancy: a 2010 update.Clin Res Hepatol Gastroenterol. 2011; 35 (3) :182-193. Haram K, Svendsen E, Abildgaard U. The HELLP syndrome: clinical issues and management. A Review.BMC Pregnancy Childbirth. 2009; 9 :8. Hepburn IS, Schade RR. Pregnancy-associated liver disorders.Dig Dis Sci. 2008; 53 (9) :2334-2358. Joshi D, James A, Quaglia A, Westbrook RH, Heneghan MA. Liver disease in pregnancy.Lancet. 2010; 375 (9714) :594-605. Mackillop L, Williamson C. Liver disease in pregnancy.Postgrad Med J. 2010; 86 (1013) :160-164. Mihu D, Costin N, Mihu CM, Seicean A, Ciortea R. HELLP syndrome - a multisystemic disorder.J Gastrointestin Liver Dis. 2007; 16 (4) :419-424. O'Brien JM, Barton JR. Controversies with the diagnosis and management of HELLP syndrome.Clin Obstet Gynecol. 2005; 48 (2) :460-477.

Use with LD and CBC in the diagnosis of HELLP Panel includes albumin; alkaline phosphatase; aspartate aminotransferase; alanine aminotransferase; bilirubin, direct; protein; bilirubin, total Use with hepatic function panel and CBC in diagnosis of HELLP

ARUP LABORATORIES | 500 Chipeta Way | Salt Lake City, Utah 84108-1221 | (800) 522-2787 | www.arupconsult.com | www.aruplab.com
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The Physician's Guide to Laboratory Test Selection and Interpretation

Reviewed by Krautscheid, Patti, MS, LCGC. Genetic Counselor, Molecular Genetics and Special Genetics Laboratories at ARUP Laboratories Lehman, Christopher M., MD. Co-Medical Director, University Hospital Clinical Laboratory; Professor of Pathology (Clinical), University of Utah Related Content Disseminated Intravascular Coagulation - DIC Hemolytic Anemias Heparin-Induced Thrombocytopenia - HIT Thrombotic Microangiopathies - TMA
Last Update: January 2013

ARUP LABORATORIES | 500 Chipeta Way | Salt Lake City, Utah 84108-1221 | (800) 522-2787 | www.arupconsult.com | www.aruplab.com
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