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A Practical Plan to Detect and Manage HELLP Syndrome ▲

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A Practical Plan to Detect and Manage HELLP Syndrome ▲ OBGMANAGEMENT BEST PRACTICES FOR DIAGNOSIS AND MANAGEMENT OF PREECLAMPSIA Baha M. Sibai, MD A practical plan to detect Professor and Chairman Department of Obstetrics and Gynecology and manage HELLP syndrome University of Cincinnati College of Medicine How to minimize the risk of serious morbidity, including tips on distinguishing HELLP from other conditions, stabilizing the patient, and managing labor, delivery, and the postpartum period. ere’s a disturbing fact: If it looks ❚ Pinning HELLP down like HELLP syndrome, and One of the best tools to identify HELLP H impairs the patient like HELLP syndrome is a healthy dose of suspicion, syndrome, it isn’t necessarily HELLP syn- since it can affect any pregnant woman at drome. A plethora of diagnostic criteria any time: antepartum, intrapartum, or from different investigators over the years within 1 week postpartum. Approximately has confused the issue of what constitutes 72% of cases are diagnosed before deliv- this syndrome—not to mention how to ery, and the rest are diagnosed during the IN THIS ARTICLE manage it. first week postpartum. A management issue has also attract- Weinstein noted that the signs and ❙ Hallmarks ed recent attention: use of corticosteroids symptoms of HELLP syndrome can occur of HELLP either antepartum to enhance maternal without clinical evidence of severe Page 55 status so that epidural anesthesia can be preeclampsia (severe hypertension and/or administered, or postpartum to improve severe proteinuria). Indeed, he reported ❙ Conditions that platelets. Such improvements are only that hypertension can be mild or absent in transient, however, and we lack definitive most patients with HELLP, and protein- heighten risk data on the benefits. uria can be mild. Page 56 One thing is certain, however. The Weinstein coined the term HELLP syn- combination of hemolysis, liver dysfunc- drome in 1982 to describe these abnormal- ❙ Cesarean delivery: tion or injury, and platelet consumption in ities in women with preeclampsia: Indications women with preeclampsia makes adverse H = hemolysis and management maternal and perinatal outcomes more EL = elevated liver enzymes Page 63 likely and leaves no room for expectant LP = low platelets management. Another obstacle to early detection: ❙ HELLP syndrome also has become a Patients may have nonspecific signs and Rare but major issue in litigation against obstetri- symptoms, none of which are diagnostic of life-threatening: cians and medical and surgical consult- classical preeclampsia. Subcapsular ants. Lawsuits usually allege misdiag- However, HELLP syndrome is most liver hematoma nosed preeclampsia, delayed delivery, or common in women who have already been Page 64 improper recognition and management of diagnosed with gestational hypertension complications. and/or preeclampsia. CONTINUED 52 OBG MANAGEMENT • April 2005 A practical plan to detect and manage HELLP syndrome ▲ TABLE 1 Hallmarks of HELLP: Hemolysis, elevated liver enzymes, and low platelets Hemolysis Diagnosis requires at least 2 of the following: ■ Abnormal peripheral smear (schistocytes, burr cells) ■ Elevated serum bilirubin (≥1.2 mg/dL) ■ Low serum haptoglobin ■ Significant drop in hemoglobin levels, unrelated to blood loss Elevated liver enzymes ■ Aspartate aminotransferase or alanine aminotransferase at least twice the upper level of normal ■ Lactate dehydrogenase at least twice the upper level of normal. This value is also elevated in severe hemolysis Low platelets ■ <100,000/mm3 IMAGE: MAURA FLYNN HELLP is more likely When to begin testing with severe hypertension In women with new-onset hypertension, Overall, the incidence of HELLP syn- order a complete blood count with platelets drome in women with gestational hyper- and liver enzyme analysis at the time of diag- FAST TRACK tension/preeclampsia increases with the nosis and serially thereafter. The frequency of HELLP syndrome severity of the condition. HELLP syn- these tests depends on the initial test results, drome also is more likely in women with severity of disease, and onset of symptoms. is more likely early-onset hypertension/preeclampsia In women without hypertension, I rec- when hypertension (before 34 weeks’ gestation). ommend obtaining the same blood tests at or preeclampsia the onset of any of the signs and symptoms is diagnosed listed in TABLE 2. before 34 weeks ❚ Making the diagnosis Assessing test results HELLP syndrome is diagnosed when all 3 Clinicians should be familiar with the of the following are present: upper limit for liver-enzyme tests in their • Hemolysis, defined as the presence of laboratory. I suggest a cutoff more than microangiopathic hemolytic anemia. twice the upper limit for a particular test. This is the hallmark of the triad. Also keep in mind that these parame- • Elevated liver enzymes (either aspar- ters are dynamic; some women will meet tate aminotransferase [AST] or alanine only some of the criteria early in the disease aminotransferase [ALT]). This compo- process. Moreover, maternal complications are nent signifies liver cell ischemia and/or substantially higher when all 3 components necrosis. are present than when only 1 or 2 are present. • Low platelet count (<100,000/mm3). TABLE 1 summarizes the laboratory Look for these clinical findings criteria for the diagnosis. Hypertension. Most women with HELLP www.obgmanagement.com April 2005 • OBG MANAGEMENT 55 ▲ A practical plan to detect and manage HELLP syndrome TABLE 2 The usual times of onset Conditions that heighten Antepartum cases. As was previously the risk of HELLP noted, HELLP syndrome usually develops before delivery, with the most frequent • Preeclampsia-eclampsia onset being before 37 weeks’ gestation Early onset (TABLE 4). During expectant management In the postpartum period, most cases devel- • Severe gestational hypertension op within 48 hours after delivery. Of these, • Early-onset hypertension, or severe approximately 90% occur in women who intrauterine growth restriction had antepartum preeclampsia that pro- • Thrombophilias gressed to HELLP syndrome in the post- • Abruptio placentae partum period. However, approximately • Nonspecific viral-syndrome-like 20% of postpartum cases develop more symptoms than 48 hours after delivery. • Right upper quadrant, epigastric, or Another important point: HELLP syn- retrosternal pain drome can develop for the first time post- • Persistent nausea or vomiting in third partum in women who had no evidence of trimester preeclampsia before or during labor. Thus, • Bleeding from mucosal surfaces it is important to educate all postpartum • Unexplained hematuria or proteinuria women to report new symptoms (listed in TABLE 3) as soon as possible. When these • Petechial hemorrhages or ecchymosis symptoms develop, evaluate the patient for both preeclampsia and HELLP syndrome. TABLE 3 Signs and symptoms Risk for life-threatening maternal complications CONDITION FREQUENCY (%) When all components of HELLP syndrome Hypertension 85 are present in a woman with preeclampsia, FAST TRACK Proteinuria 87 the risk of maternal death and serious HELLP syndrome Right upper quadrant 40–90 maternal morbidities increases substantial- or epigastric pain ly (TABLE 5). The rate of these complica- sometimes appears Nausea or vomiting 29–84 tions depends on gestational age at onset, for the first time Headaches 33–60 presence of associated obstetric complica- postpartum Visual changes 10–20 tions (eclampsia, abruptio placentae, peri- Mucosal bleeding 10 partum hemorrhage, or fetal demise) or in women who had preexisting conditions (lupus, renal disease, Jaundice 5 no evidence chronic hypertension, or type 1 diabetes). of preeclampsia Abruptio placentae increases the risk of before or during syndrome have hypertension. In 15% to disseminated intravascular coagulopathy 50% of cases, the hypertension is mild, but (DIC), as well as the need for blood trans- labor it may be absent in 15%. fusions. Proteinuria. Most patients also have pro- Marked ascites (>1 L) leads to higher rates teinuria by dipstick (≥1+). Proteinuria may of cardiopulmonary complications. be absent in approximately 13% of women with HELLP syndrome, although Differential diagnosis they will likely have many of the symp- When diagnosing HELLP syndrome, con- toms reported by women with severe firm or exclude the conditions listed in preeclampsia. TABLE 6, since the presenting symptoms TABLE 3 lists the signs and symptoms and clinical and laboratory findings in to be expected in these patients, along with women with HELLP syndrome overlap their frequency. those of several microangiopathic disor- 56 OBG MANAGEMENT • April 2005 ▲ A practical plan to detect and manage HELLP syndrome sudden deterioration in maternal and fetal TABLE 4 conditions, patients should be hospitalized Usual times of onset* and observed in a labor and delivery unit. Initially, assume the patient has severe RELATION TO DELIVERY PERCENTAGE preeclampsia and treat her with intra- Antepartum 72 venous magnesium sulfate to prevent con- Postpartum 28 vulsions and antihypertensive medications ≤48 hours 80 as needed to keep systolic blood pressure >48 hours 20 below 160 mm Hg and diastolic blood GESTATIONAL AGE (WEEKS) PERCENTAGE pressure below 105 mm Hg. 17–20 2 Blood tests should include: • complete blood count with platelet count, 21–27 10 • peripheral smear evaluation, 28–36 68 • serum AST, >37 20 • lactate dehydrogenase, * Based on 700 cases • creatinine, • bilirubin, and TABLE 5 • coagulation studies. Maternal complications These tests help confirm the diagnosis
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