HELLP Syndrome Diagnosis

Occurrence:

(1) The syndrome may present during the second trimester, third trimester, at term or in the postpartum period.

(2) The disorder may occur at any maternal age and may occur in both primi- and multi-gravida mothers.

 

Symptoms:

(1) malaise

(2) epigastric or right upper quadrant pain

(3) nausea or vomiting

(4) viral infection-like symptoms

(5) headache (especially if hypertension present)

 

Pre-eclampsia or eclampsia is often present but may be absent.

(1) features of pre-eclampsia: hypertension, proteinuria and edema

(2) features of eclampsia: features of pre-eclampsia +  seizures

 

Diagnostic features:

(1) hemolysis ("H")

(2) elevated liver enzymes ("EL")

(3) low platelet count ("LP")

 

Hemolysis:

(1) Schistocytes and other microangiopathic findings are present, indicating intravascular hemolysis

(2) If intravascular hemolysis is sufficiently severe, then the patient will develop anemia (lower limit of reference range for hemoglobin in young adult women is 11.7 g/dL) an elevated bilirubin, increased serum LDH and decreased serum haptoglobin.

 

Elevated liver enzymes:

(1) The AST (SGOT) increases is greater than the mean of the reference range + 3SD

(2) Since most laboratories use the reference range as the mean +/- 2 SD (to include 95% of the "normal" population) and the mean = ((lower end) + (upper end)) / 2:

 

3 SD above the mean =

= (1.25 * (upper end of reference range)) – (0.25 * (lower end of the reference range))

 

Low platelet count:

(1) classically < 100,000 per µL

(2) A platelet count > 100,000 per µL may indicate impending onset if there has been a significant drop from a higher value.

(3) A bone marrow biopsy shows increased megakaryocytes.

 

Differential diagnosis:

(1) DIC (fibrin split products, low fibrinogen)

(2) fatty liver of pregnancy

(3) vasculitis

(4) ITP

(5) TTP/HUS

 

Diagnosis:

(1) If all the features are present, the diagnosis of HELLP may be made.

(2) The features in the absence of hemolysis is referred to as the ELLP syndrome.

(3) Schistocytes without anemia, rising AST and/or falling platelet count may indicate impending onset of the syndrome.

 

Complications:

(1) development of DIC, which may be associated with occurrence of obstetrical complications

(2) acute renal failure (increased risk if HELLP onset in postpartum period)

(3) pulmonary edema (increased risk if HELLP onset in postpartum period)

(4) ARDS

(5) abruptio placentae

(6) hepatocellular necrosis

(7) ascites

(8) subcapsular liver hematoma, which may rupture with intra-abdominal hemorrhage

(9) retinal detachment

(10) cerebral edema

 

Management:

(1) Early recognition allows for prompt management.

(2) The patient should be placed on strict bed rest, preferably in the left lateral decubitus position.

(3) The patient may benefit from transfer to a larger facility with greater expertise and resources.

(4) Fetal monitoring should be performed. Amniocentesis may be indicated to assess fetal maturity.

(5) The fetus should be delivered if possible. Corticosteroids may be given to enhance fetal lung development.

(6) Magnesium sulfate is given to help prevent convulsions if pre-eclampsia or eclampsia are present.

(7) Antihypertensive medications include a beta-blocker or hydralazine.

(8) Intravenous fluid infusion needs to be done carefully. This can help counter the plasma volume contraction associated with the disease but, if done excessively, may contribute to peripheral and pulmonary edema. Fluids include crystalloids, albumin, and fresh frozen plasma (FFP).

(9) Plasma exchange may be done in severe cases in the postpartum period.

(10) Dexamethasone may be used in severe cases.

(11) Low-dose aspirin or heparin are sometimes given.

(12) Transfusion of platelets products may be done if the thrombocytopenia becomes severe. It is important to have excluded DIC if platelet transfusion is being considered.

(13) If renal failure develops then dialysis should be considered.

 

The rate of recurrence in future pregnancies is low (about 3%).