Elsevier

Clinics in Perinatology

Volume 31, Issue 4, December 2004, Pages 807-833
Clinics in Perinatology

Diagnosis and management of hemolysis, elevated liver enzymes, and low platelets syndrome

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Terminology and diagnosis

A review of the literature by Sibai et al [6] revealed considerable variety in the terminology, reported incidence, reported cause, diagnosis, and management of HELLP syndrome. Goodlin considered it an early form of severe pre-eclampsia and labeled it a great imitator, equating it with impending edema, proteinuria, hypertension gestosis type B [2]. Weinstein [5] considered it a “unique variant” of pre-eclampsia, whereas MacKenna et al [7] considered it misdiagnosed pre-eclampsia. Conversely,

Clinical presentation

In the series reported by Sibai et al [6], patients with HELLP syndrome were significantly older (mean age 25 years) than patients with severe pre-eclampsia/eclampsia without features of HELLP syndrome (mean age 19 years). The incidence of the syndrome was significantly higher in the white population and among multiparous patients. The incidence of HELLP syndrome is also higher in pre-eclamptic patients with conservative management of their disease. Coincidentally, medial complications (notably

Initial management

The clinical course of women with HELLP syndrome is characterized by usually progressive and sometimes sudden deterioration in maternal and fetal conditions. Therefore, patients with suspected diagnosis of HELLP syndrome should be hospitalized immediately and observed in a labor and delivery unit. The first priority is to assess and stabilize maternal condition, particularly coagulation abnormalities (Box 2). Patients with HELLP syndrome who are remote from term should be referred to a tertiary

Conservative management

Van Pampus et al [47] described the clinical progress and maternal outcome of the HELLP and ELLP (findings of HELLP syndrome, but without evidence of hemolysis) syndromes in 127 patients managed in the Academic Medical Center in Amsterdam between 1984 and 1996 with a live fetus in utero. The patients were treated by temporizing management, including the use of antihypertensives and magnesium sulfate. The predominant indication for terminating pregnancy was fetal distress or fetal death, not

Delivery management

When HELLP syndrome develops at or beyond 34 weeks' gestation, or when there is evidence of fetal lung maturity or fetal or maternal jeopardy before that time, delivery is the definitive therapy. Without laboratory evidence of DIC and absent fetal lung maturity, the patient can be given steroids to accelerate fetal lung maturity and be delivered 48 hours later. Maternal and fetal conditions should be assessed continuously during this time (Fig. 2).

The presence of HELLP syndrome is not an

Hepatic manifestations

The authors reported hepatic imaging findings in selected patients with HELLP syndrome and correlated these findings with the severity of concurrent clinical and laboratory abnormalities [50]. Of the 34 patients evaluated in the study, 16 patients (47%) had abnormal hepatic imaging results. The most common CT abnormalities were subcapsular hematoma of the liver (n = 13) and intraparenchymal hemorrhage (n = 6). An MRI of an unruptured subcapsular hematoma of the liver is depicted in Fig. 3.

Postpartum management

The HELLP syndrome may develop ante partum or post partum. An analysis of 442 cases by Sibai et al [59] revealed that 309 (70%) had evidence of the syndrome ante partum, and 133 (30%) developed the manifestations post partum. Four maternal deaths occurred, and morbidity was frequent (Table 6). In the postpartum period, the time of onset of the manifestations ranged from a few hours to 7 days, with most developing within 48 hours post partum. Patients in this group are at increased risk for the

Maternal counseling

Pregnancies complicated by HELLP syndrome may be associated with life-threatening complications for both the mother and her infant. Therefore, clinicians should be able to answer questions about subsequent pregnancy outcome and long-term prognosis. Women with a history of HELLP syndrome are at increased risk for all forms of pre-eclampsia in subsequent pregnancies (Table 7). In general, the rate of pre-eclampsia in subsequent pregnancies is approximately 20%, with significantly higher rates if

Summary

Pregnancies complicated by HELLP syndrome require a well-formulated management plan. The development of this syndrome after 34 weeks' gestation or with documentation of maternal or fetal compromise is an indication for delivery. Vaginal delivery can be accomplished in most cases; however, if cesarean section is required, subfascial drains and preoperative platelet transfusion for platelet counts of less than 40,000/mm3 can reduce the incidence of complications. AFLP, TTP, or HUS may present

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