Look for these clinical findings
Hypertension. Most women with HELLP syndrome have hypertension. In 15% to 50% of cases, the hypertension is mild, but it may be absent in 15%.
Proteinuria. Most patients also have proteinuria by dipstick (≥1+). Proteinuria may be absent in approximately 13% of women with HELLP syndrome, although they will likely have many of the symptoms reported by women with severe preeclampsia.
TABLE 3 lists the signs and symptoms to be expected in these patients, along with their frequency.
TABLE 3
Signs and symptoms
| CONDITION | FREQUENCY (%) |
|---|---|
| Hypertension | 85 |
| Proteinuria | 87 |
| Right upper quadrant or epigastric pain | 40–90 |
| Nausea or vomiting | 29–84 |
| Headaches | 33–60 |
| Visual changes | 10–20 |
| Mucosal bleeding | 10 |
| Jaundice | 5 |
The usual times of onset
Antepartum cases. As was previously noted, HELLP syndrome usually develops before delivery, with the most frequent onset being before 37 weeks’ gestation ( TABLE 4).
In the postpartum period, most cases develop within 48 hours after delivery. Of these, approximately 90% occur in women who had antepartum preeclampsia that progressed to HELLP syndrome in the postpartum period. However, approximately 20% of postpartum cases develop more than 48 hours after delivery.
Another important point: HELLP syndrome can develop for the first time postpartum in women who had no evidence of preeclampsia before or during labor. Thus, it is important to educate all postpartum women to report new symptoms (listed in TABLE 3) as soon as possible. When these symptoms develop, evaluate the patient for both preeclampsia and HELLP syndrome.
TABLE 4
Usual times of onset*
| RELATION TO DELIVERY | PERCENTAGE |
| Antepartum | 72 |
| Postpartum | 28 |
| ≤48 hours | 80 |
| >48 hours | 20 |
| GESTATIONAL AGE (WEEKS) | PERCENTAGE |
| 17–20 | 2 |
| 21–27 | 10 |
| 28–36 | 68 |
| >37 | 20 |
| * Based on 700 cases | |
Risk for life-threatening maternal complications
When all components of HELLP syndrome are present in a woman with preeclampsia, the risk of maternal death and serious maternal morbidities increases substantially (TABLE 5). The rate of these complications depends on gestational age at onset, presence of associated obstetric complications (eclampsia, abruptio placentae, peripartum hemorrhage, or fetal demise) or preexisting conditions (lupus, renal disease, chronic hypertension, or type 1 diabetes).
Abruptio placentae increases the risk of disseminated intravascular coagulopathy (DIC), as well as the need for blood transfusions.
Marked ascites (>1 L) leads to higher rates of cardiopulmonary complications.
TABLE 5
Maternal complications
| COMPLICATION | FREQUENCY (%) |
|---|---|
| Death | 1 |
| Adult respiratory distress syndrome | 1 |
| Laryngeal edema | 1–2 |
| Liver failure or hemorrhage | 1–2 |
| Acute renal failure | 5–8 |
| Pulmonary edema | 6–8 |
| Pleural effusions | 6–10 |
| Abruptio placentae | 10–15 |
| Disseminated intravascular coagulopathy | 10–15 |
| Marked ascites | 10–15 |
Differential diagnosis
When diagnosing HELLP syndrome, confirm or exclude the conditions listed in TABLE 6, since the presenting symptoms and clinical and laboratory findings in women with HELLP syndrome overlap those of several microangiopathic disorders that can develop during pregnancy and/or postpartum. In some women, preeclampsia may be superimposed on one of these disorders, further confounding an already difficult differential diagnosis.
Because of the remarkably similar clinical and laboratory findings of these diseases, make every effort to achieve an accurate diagnosis, since management and outcomes may differ among these conditions.
TABLE 6
Differential diagnosis
|
Initial Management
Hospitalize the patient
Because HELLP syndrome usually is characterized by progressive and sometimes sudden deterioration in maternal and fetal conditions, patients should be hospitalized and observed in a labor and delivery unit.
Initially, assume the patient has severe preeclampsia and treat her with intravenous magnesium sulfate to prevent convulsions and antihypertensive medications as needed to keep systolic blood pressure below 160 mm Hg and diastolic blood pressure below 105 mm Hg.
Blood tests should include:
- complete blood count with platelet count,
- peripheral smear evaluation,
- serum AST,
- lactate dehydrogenase,
- creatinine,
- bilirubin, and
- coagulation studies.
These tests help confirm the diagnosis and check for the presence of DIC, massive hemolysis, severe anemia, or renal failure.
The first priority is to assess the patient for the presence of cardiovascular complications, signs of liver hematoma or hemorrhage, and abruptio placentae. If any is present—particularly hypotension, hypovolemia, DIC, or pulmonary edema—make every effort to stabilize the maternal condition.
Can delivery wait 48 hours for corticosteroids?
Evaluate fetal status by heart rate monitoring or biophysical profile, and confirm gestational age. Then decide whether delivery is indicated or can be delayed for 48 hours so that corticosteroids can be given.
No room for expectant management. Do not consider expectant management in women with true HELLP syndrome. Delivery can only be delayed for a maximum of 48 hours—and only when both mother and fetus are stable, at 24 to 34 weeks’ gestation, and awaiting the benefit of corticosteroids.
Corticosteroid dosing. My practice is to give 2 doses of either betamethasone 12 mg intramuscularly every 12 hours or dexamethasone 12 mg intravenously every 12 hours. This is to improve maternal status, at least temporarily.