HELLP Syndrome Cause, Definition, Hellp in Pregnancy, Symptoms and Treatment

What is HELLP Syndrome?

HELLP Syndrome stands for Hemolysis Elevated Liver enzymes Low Platelets. The term HELLP syndrome was coined to emphasize the dangerous combination of disseminated intravascular coagulation and liver damage in severe preeclampsia. HELLP syndrome occurs when liver involvement is associated with hemolysis and low platelets. This is a severe variant of preeclampsia.

HELLP Syndrome Epidemiology

HELLP syndrome affects about 4-12% of those with Preeclampsia or Eclampsia and is commoner in Multigravidae (Women that has had more than one pregnancy). Though Preeclampsia is commoner in Primigravidas (women that are pregnant for the first time), whenever a Multigravida is found to have Preeclampsia, her risk of having HELLP syndrome is higher than a Primigravida. If you once had HELLP syndrome, the chance of you having it again is about 20%.

HELLP Syndrome Pathophysiology

HELLP syndrome is a complication of Preeclampsia as well as Eclampsia and may occur in the absence of hypertension. The patient may present with epigastric pain or right upper quadrant pain. Whenever there is severe pain with tender hepatomegaly, it may suggest subcapsular hematoma of the liver. The management approach is to stabilize the mother and deliver the baby; before delivery, there is need to give high doses of steroids as well as platelet transfusion. When protein excretion exceeds 3 g in 24 hours, the circulating albumin is likely to fall (Nephrotic syndrome) and this increases the risk of pulmonary edema. Lactate dehydrogenase levels will increase in the presence of hemolysis.

HELLP Symptoms

1. Epigastric pain (Chest pain) or right upper quadrant pain
2. Nausea
3. Vomiting

HELLP Syndrome Signs

1. Right upper quadrant tenderness
2. Rising levels of AST and ALT enzymes of the liver
3. Acute renal failure
4. Disseminated intravascular coagulation (DIC)

HELLP Syndrome Diagnosis

1. HELLP is diagnosed when there is rising liver enzymes (Aspartate aminotransferase, AST and Alanine aminotransferase, ALT).
2. Falling hemoglobin level
3. Falling Platelets count

These signs used in diagnosis are an indication for immediate delivery as these women are at high risk of developing Eclampsia and liver failure.

HELLP Syndrome Treatment

1. Management of HELLP syndrome is to stabilize coagulation (this is the reason why platelet transfusion is important)
2. Assess fetal well-being
3. Consider the need for delivery. It is generally considered that delivery is appropriate for moderate to severe cases of HELLP, but management may be more conservative (with close monitoring) if it is a mild case. Postpartum vigilance is required for at least 48 hours.

HELLP Labs (Investigations and their importance)

1. Liver function tests shows elevated alanine and aspartate transaminases in the HELLP syndrome
2. A platelet count above 100 x 109/L is unlikely to be associated with other coagulation abnormalities.
3. A clotting profile (clotting time, prothrombin time, partial thromboplastin time, fibrinogen) level can be done when there is severe thrombocytopenia or clinical suspicion of disseminated intravascular coagulation (DIC).

HELLP Syndrome Complications

1. Acute renal failure
2. Disseminated intravascular coagulation (DIC)
3. Increased risk of placental abruption
4. Risk of hepatic hematoma (rare)
5. Increase incidence of Hepatic rupture leading to profuse intraperitoneal bleeding