HELLP SYNDROME

Case Conference 10/16/08

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31 y.o. caucasian female, gravida 1. Estimated gestational age: 26 3/7 weeks Presented to the local ER with a seizure at home. BP 158-194/117-133 Plt 32,000 ALT 339 AST 770 Cr 0.99 Treated with multiple doses of labetalol and given magnesium sulfate and betamethasone prior to transfer.

Labs
HGB 8.5L Plt 30 Uric acid 10.5 Mg 7.8 LDH 1073

Initial Evaluation
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Patient is very ill with HELLP and eclampsia. Recommend proceeding with delivery after platelets are available for transfusion. Delivery by cesarean section is recommended given patient's current condition, remote from delivery, and fetal intolerance of labor. NICU Consult Seizure Prophylaxis - Magnesium Sulfate at 2 grams/hour Laboratory Assessment, CBC, T&S, AST, ALT, Mg, Electrolytes, INR, APTT Continue Transfuse platelets as soon as possible (pheresed, leukoreduced, irradiated)

Post-procedure Diagnoses l 1. ECLAMPSIA l 2. HELLP (HEMOLYTIC ANEMIA/ELEV LIVER ENZYMES/LOW PLATELETS IN PREGNANCY) Procedure l 1. CESAREAN DELIVERY

F/u Labs
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HGB -HCT -PLT 154 AST 43H ALT 68H CREATININE Mg 5 14

8.1L 23.9L 117L 44H 78H --

24.5 87L 100H 130H 0.67

8.5L 10.8L 30.5L -51L 133H 140H

Hypercoagulable Workup
Anticardiolipin Abs neg l F-r II gene mutation l F-r V Leiden l MTHFR C677T mutation (hyperhomocysteinemia) l Protein C
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Definition
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Syndrome characterized by hemolysis with microangiopatic blood smear, elevated liver enzymes, and a low platelet count. l Develops in 1 in 1000 pregnancies; in 10- 20% of cases of severe with severe preeclampsia / eclampsia l 70% - prior to delivery; may occur up to 7 days after delivery. l HTN and proteinuria present in 85%

Pathogenesis
Multiorgan dysfunction arising from endothelial abnormality with secondary fibrin deposition and organ hypoperfusion l Microangiopatic hemolytic anemia l Consumptive coagulopathy
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Clinical Presentation
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Abdominal pain Epigastric or RUQ tenderness Nausea, vomiting HTN Proteinuria MAHA Plt < 100 LDH > 600 AST > 70

Diff Dx
Acute fatty liver of pregnancy l TTP-HUS l Hepatitis l ITP l Antiphospholipid syndrome
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Management
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Delivery if:

> or= 34 weeks of gestation Fetal distress Severe maternal disease

Keep BP < 160/105 l Mg sulfate IV to prevent Sz l Steroids if < 34 weeks

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Complications
Hepatic infarction l Subcapsular hepatic hematoma (1%) and rupture l DIC 21% l Abruption placentae 16% l Acute renal failure 8% l Pulmonary edema 6% l Retinal detachment 1%
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