Pamela G. Blake

The natural history of HELLP syndrome: patterns of disease progression and regression.

Despite much recent interest in HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count), there is little published information about the natural history of this unique form of severe preeclampsia-eclampsia. The time course and pattern of laboratory abnormalities for 158 patients with HELLP syndrome managed in a single tertiary referral center between 1980 and 1989 were studied retrospectively. Despite considerable patient variation, most gravid women with HELLP syndrome had decreasing platelet counts until 24 to 48 hours after delivery. Conversely, lactate dehydrogenase concentrations usually peaked 24 to 48 hours post partum. In all patients who recovered, a platelet count greater than 1,000,000/mm3 was spontaneously achieved by the sixth postpartum day or within 72 hours of platelet nadir. An upward trend in platelet count and a downward trend in lactate dehydrogenase concentrations should be apparent in patients without complications by the fourth postpartum day. These data provide baseline information against which the course of individual patients can be compared and the infrequent, atypical case identified for interventive therapy.

The spectrum of severe preeclampsia: Comparative analysis by HELLP (hemolysis, elevated liver enzyme levels, and low platelet count) syndrome classification☆☆☆★

OBJECTIVE This study was undertaken to explore the spectrum of maternal disease with a triple classification system of HELLP (hemolysis, elevated liver enzyme levels, and low platelet count) syndrome and compare these classes with severe preeclampsia without HELLP syndrome. STUDY DESIGN In this retrospective analytic study the pregnancies of 777 patients with class 1, 2, or 3 HELLP syndrome were compared and contrasted with those of 193 women with severe preeclampsia but without HELLP syndrome. RESULTS Eclampsia, epigastric pain, nausea and vomiting, significant proteinuria, major maternal morbidity, and stillbirth increased as HELLP syndrome worsened from class 3 to class 1. In contrast, headache and diastolic hypertension were more common among the significantly heavier patients with severe preeclampsia without HELLP syndrome. Approximately half of pregnancies complicated by class 1 HELLP syndrome exhibited significant maternal morbidity, compared with only 11% of those complicated by severe preeclampsia without HELLP syndrome. Although a significant trend was apparent in increasing levels of lactate dehydrogenase, aspartate aminotransferase, and uric acid as HELLP syndrome worsened, there was considerable variation within groups. CONCLUSION Laboratory and clinical indices of disease severity in patients with severe preeclampsia or eclampsia generally were highest with class 1 HELLP syndrome and were lowest when HELLP syndrome was absent. Class 3 HELLP syndrome is considered a clinically significant transitional group.

pregnancy Complicated by Preeclampsia-eclampsia With the Syndrome of Hemolysis, Elevated Liver Enzymes, and Low Platelet Count : how Rapid is Postpartum Recovery?

The rapidity of postpartum disease recovery for severe preeclampsia associated with hemolysis, elevated liver enzymes, and low platelet count (HELLP syndrome) has not been well studied. Between January 1980 and March 1989, 158 pregnancies with preeclampsia-eclampsia complicated by HELLP syndrome were managed at the University of Mississippi Medical Center. The 70 patients with platelet nadir below 50,000/microL (class 1 HELLP syndrome) required as long as 11 days for all members to achieve a platelet recovery concentration of more than 100,000/microL, whereas all 88 gravidas with platelet nadir between 50,000-100,000/microL (class 2 HELLP syndrome) exceeded this platelet concentration by the sixth postpartum day, a statistically significant difference (P less than .0001). The interval between delivery and the onset of diuresis (mean +/- SD) was significantly longer in class 1 than in class 2 patients with milder disease (22.7 +/- 18.9 compared with 15.9 +/- 11.1 hours). Significantly more postpartum days were required in class 1 than in class 2 HELLP parturients for the lactic dehydrogenase (LDH) concentration to decrease below 500 IU/L (4.2 +/- 4.9 compared with 3.2 +/- 2.7 days). No women in the class 2 group required plasma exchange therapy to effect disease arrest and reversal, but 11 of 58 severely ill women in class 1 were treated with this modality. We conclude that the platelet count and LDH serum concentration, as indicators of HELLP severity and recovery, are clinically useful tools and that a more protracted postpartum recovery period should be expected for progressively severe expressions of HELLP syndrome.

The recurrence risk of the syndrome of hemolysis, elevated liver enzymes, and low platelets (HELLP) in subsequent gestations

OBJECTIVE Although it is an important clinical issue, accurate prediction of recurrence risk for the syndrome of hemolysis, elevated liver enzymes, and low platelets (HELLP) has been problematic because of limited patient experience. This study was undertaken to determine the likelihood that this form of severe preeclampsia-eclampsia or any other hypertensive disorder would occur in a subsequent pregnancy. STUDY DESIGN An extensive retrospective analysis of medical records and patient follow-up regarding subsequent pregnancy outcome were undertaken for the 481 patients with HELLP syndrome managed at this tertiary medical center between Jan. 1, 1980, and Oct. 30, 1991. The Mississippi three-class system was used to define severity of disease on the basis of the lowest observed perinatal platelet count (class 1 < or = 50,000/microliters, class 2 > 50,000/microliters to < or = 100,000/microliters, and class 3 > 100,000/microliters to < or = 150,000/microliters). RESULTS Subsequent gestations (n = 195) occurred in 122 of 481 patients. Evaluable data were available for analysis in 161 of 195 possible pregnancies. Seventy-eight (48%) pregnancies were complicated by some type of hypertensive disorder, 44 (27%) of which had class 1, 2, or 3 HELLP syndrome. Non-HELLP preeclampsia-eclampsia was detected in 25 subsequent gestations (15%). Thus the total frequency of preeclampsia was 69 in 161 (43%). If the data for class 3 HELLP are completely excluded from the analysis, 81 subsequent evaluable and viable gestations were identified, 19 pregnancies with preeclampsia-eclampsia (23%) and 15 patients with HELLP syndrome (19%), for a total recurrence rate of 42%. Subsequent HELLP gestations were frequently delivered abdominally (64%) on average 2 weeks later than the index pregnancy (32.6 +/- 5.0 weeks versus 34.7 +/- 5.3 weeks). Delivery at < 32 weeks conferred a high risk (61%) for a similar preterm delivery in a subsequent gestation. CONCLUSION The risk of recurrence of the HELLP syndrome in our population is 19% to 27%. When data from all pregnancies with all forms of preeclampsia are considered, the risk of recurrence for any type of preeclampsia-eclampsia is 42% to 43%. A previous preterm delivery is a very high risk factor for recurrence of prematurity with preeclampsia-eclampsia.

Better maternal outcomes are achieved with dexamethasone therapy for postpartum HELLP (hemolysis, elevated liver enzymes, and thrombocytopenia) syndrome ☆ ☆☆ ★ ★★

OBJECTIVE Our purpose was to determine whether the routine initiation of dexamethasone therapy in patients with postpartum HELLP (hemolysis, elevated liver enzymes, and thrombocytopenia) syndrome produces specific and general therapeutic benefits. STUDY DESIGN In this retrospective, analytic study the puerperal courses of 43 women with postpartum HELLP syndrome who were treated with dexamethasone were compared with those of 237 similar patients who did not receive corticosteroids. Dexamethasone 10 mg intravenously at 12-hour intervals was given until disease remission was noted in treated patients, at which time up to two additional 5 mg intravenous doses were given at 12-hour intervals. RESULTS The two patient groups were similar in regard to mode of delivery, gestational age, parity, and frequency of eclampsia. Compared with control subjects, dexamethasone-treated postpartum patients were more ill with significantly higher (p < 0.05) admission mean arterial blood pressure, higher serum uric acid level, and severe proteinuria. Dexamethasone administration was associated with a more rapid normalization of platelet counts and lactic dehydrogenase values. Most impressive was a clinically significant reduction of indicated transfusion and respiratory therapy, invasive hemodynamic monitoring, infectious or bleeding-related morbidity, and length of postpartum hospital course. CONCLUSIONS Patients who received dexamethasone for postpartum-onset HELLP syndrome experienced a shorter disease course, faster recovery, less morbidity, and less need for other interventionist therapy compared with patients with HELLP syndrome who did not receive dexamethasone.

Early risk assessment of severe preeclampsia: Admission battery of symptoms and laboratory tests to predict likelihood of subsequent significant maternal morbidity

OBJECTIVE This study was undertaken to investigate the utility of an admission battery of findings and laboratory data in the discrimination of patients with severe preeclampsia with or without HELLP (hemolysis, elevated liver enzyme levels, and low platelet count) syndrome at high risk for development of significant maternal morbidity. STUDY DESIGN The clinical and laboratory findings at hospital admission for 970 patients with severe preeclampsia with or without HELLP syndrome were studied retrospectively to develop parameters associated with low, moderate, and high risks for the subsequent development of significant maternal morbidity involving the hematologic and coagulation, cardiopulmonary, and hepatorenal systems. RESULTS Nausea and vomiting and epigastric pain are independent risk factors for complicated severe preeclampsia. Results of a panel of tests with values including lactate dehydrogenase level >1400 IU/L, aspartate aminotransferase level >150 IU/L, alanine aminotransferase level >100 IU/L, uric acid level >7.8 mg/dL, serum creatinine level >1.0 mg/dL, and 4+ urinary protein by dipstick can be used to discriminate the patient at high risk for significant maternal morbidity. Concentrations of lactate dehydrogenase, aspartate aminotransferase, and uric acid above these cut points have the strongest predictive value and are risk additive with worsening thrombocytopenia. CONCLUSION The presence of nausea and vomiting, epigastric pain, or both in association with admission laboratory values that are in excess of the cutoffs for lactate dehydrogenase, aspartate aminotransferase, and uric acid concentrations or for all 6 tests is predictive of high risk of morbidity for the patient with severe preeclampsia. These factors are independent of and additive with the rising maternal risk associated with decreasing platelet count.

Postpartum plasma exchange for atypical preeclampsia-eclampsia as HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome

OBJECTIVE Our purpose was to investigate the postpartum use of plasma exchange in patients considered to have atypical preeclampsia-eclampsia manifested as persistent HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome with or without evidence of other organ injury. STUDY DESIGN During a 10-year period, 18 patients with HELLP syndrome were treated post partum with single or multiple plasma exchange with fresh-frozen plasma. Each patient was entered into the clinical trial either because of persistent evidence of atypical preeclampsia-eclampsia as HELLP syndrome > 72 hours after delivery (group 1) or with evidence of worsening HELLP syndrome at any time post partum in association with single- or multiple-organ injury (group 2). All procedures were performed with the IBM 2997 Cell Separator (IBM, Cobe Laboratories, Inc., Lakewood, Colo.) system. Maternal and perinatal outcomes were the main outcomes studied. RESULTS In the absence of other disease conditions, the 9 patients in group 1 with persistent postpartum HELLP syndrome complicated only by severe clinical expressions of preeclampsia-eclampsia responded rapidly to one or two plasma exchange procedures with few complications and no maternal deaths. In contrast, in the 9 patients of group 2 with HELLP syndrome presentations complicated by other organ disease, the response to plasma exchange was variable and there were two deaths in this group. CONCLUSION The current series of patients details the successful postpartum application of plasma exchange therapy for unremitting HELLP syndrome but reveals that a uniformly positive response to this therapy will not always be observed when there is additional single or multiple organ injury.

Vaginal birth after cesarean delivery: Are there useful and valid predictors of success or failure?

OBJECTIVE Before parturition are there useful and valid predictors of successful or unsuccessful vaginal birth after previous cesarean birth that could be used to enhance the obstetric care of a patient and her pregnancy? STUDY DESIGN The clinical course and outcome of all patients who attempted vaginal birth after cesarean delivery at one level III center during 1989 were evaluated to identify factors prognostic of a successful or unsuccessful patient group; use of this information in stepwise logistic regression and cluster analysis was disappointing. RESULTS No single criterion or optimal clusters of factors were found and no equation achieved greater than 75% predictability of outcome with acceptable sensitivity and specificity. CONCLUSIONS Before parturition prediction of outcome of vaginal birth after cesarean delivery is tenuous regardless of past obstetric history or recent clinical parameters. Thus it seems appropriate to encourage a trial of labor in almost all patients with a prior low-segment uterine incision (transverse or vertical) unless there is a strong physician or patient-derived contraindication to such an undertaking.

Corticosteroids for the Enhancement of Fetal Lung Maturity: Impact on the Gravida with Preeclampsia and the HELLP Syndrome

Summary: This study was undertaken to determine maternal impact of corticosteroids administered for the promotion of fetal lung maturity in mothers with the HELLP syndrome. Twenty‐seven of 427 women with the HELLP syndrome treated between 1980–1991 received a full course of steroids prior to preterm delivery. This group was compared to 27 matched control patients with the HELLP syndrome who received no corticosteroids. Subjects were matched for maternal age, race, sex of the fetus, and severity of the HELLP syndrome. The antepartum platelet count stabilized or increased in 25 of 27 steroid‐treated women in contrast to 0 of 15 control women (p <0.00001). In comparison to control patients, LDH serum concentrations in steroid‐treated patients stabilized or decreased and the SGOT/AST and SGPT/ALT stabilized or decreased during therapy (p < 0.005). The interval from delivery to platelet nadir in patients with Class III HELLP syndrome was shorter in the steroid‐treated group (p<0.008) than in untreated patients.

The interrelationship of eclampsia, HELLP syndrome, and prematurity: cofactors for significant maternal and perinatal risk

Objective To determine if there are differences between mothers and fetuses in eclamptic pregnancies with or without concurrent HELLP syndrome.