HELLP SYNDROME- Introduction, Key Facts & Statistics, Risk Factors, Causes, Signs & Symptoms, Categorization, Diagnostic Procedures & Tests, Differential Diagnosis, Treatment and Management, Complications, Prevention

Introduction

HELLP is an acronym for Hemolysis, Elevated Liver enzymes and Low Platelets. HEELP syndrome is a potentially life-threatening complication that is considered to be a variant of pre-eclampsia.

 

 Key Facts & Statistics

Key facts and statistics that surround HELLP syndrome

  • HELLP syndrome is a severe complication that results due to pregnancy. It co-exists in about 70% of cases with pre-eclampsia.
  • It occurs in about 1 to 2 out of 1,000 pregnancies.
  • In 1982, Dr. Loius Weinstein named it after its characteristic features. HEELP:
  1. H- Hemolysis. This is a process whereby erythrocytes, otherwise known as red blood cells break down.
  2. EL- Elevated liver enzymes.
  3. LP- Low platelet count (thrombocytopenia).
  • Death can occur in almost 25% of cases.
  • Mostly, death is due to liver rupture or stroke (as a result cerebral edema or cerebral hemorrhage).
  • It is estimated that in the US about 45,000 pregnant women will develop HELLP syndrome.
  • It develops during the later stages (third stages) of pregnancy (between the 26th and the 40th weeks of gestation). It may also develop post-partum.

 

 Risk Factors

What are the risk factors for HELLP syndrome?

  • Maternal age. Mother being older than 35 years of age.
  • Obesity. Being excessively overweight.
  • Having history of:
  1.  Diabetes.
  2.  Renal disease.
  3.  Chronic high blood pressure.
  4.  Preeclampsia.
  5.  Multiple births.

 

 Causes

What are the causes of HELLP syndrome?

The exact cause(s) of pre-eclampsia are not yet established.

 

 Signs & Symptoms

What are the signs and symptoms of HELLP syndrome?

  • Fatigue.
  • Retention of fluid.
  • Headache.
  • Excess weight gain.
  • Vision changes such as double vision and blurred vision.
  • Nausea and vomiting.
  • Abdominal pain and chest pain.
  • Bleeding (nosebleeding or any other form of bleeding that does not stop).
  • Shortness of breath (dyspnea).

 

Measurable signs and symptoms to look for:

  •  High blood pressure.
  •  Proteinuria (protein in the urine).
  •  Abnormal results of lab blood tests such as elevation in liver enzymes, low platelets and hemolysis.

 

Categorization

How is HELLP syndrome classified?

It is classified into 3 groups. These are:

Class I (severe thrombocytpenia)

  •  AST ≥ 70 IU/L
  •  LDH ≥ 600 IU/L
  •  Platelets ≤ 50,000 /uL

 

Class II (moderate thrombocytopenia)

  • AST ≥50 IU/L
  •  LDH ≥600 IU/L
  •  Platelets >50,000 ≤100,000 /uL

 

Class III (mild thrombocytopenia)

  • AST ≥40 IU/L
  •  LDH>600 IU/L
  •  Platelets> 100,000 ≤ 150,000 /uL

 

AST is aspartate aminotransferase while LDH is lactate dehydrogenase. AST is made by the liver in very low amounts and high levels indicate damage to the liver.

LDH is involved in production of energy by body cells. Due to cellular damage or destruction, LDH is released into the blood from cells. Higher than normal levels are suggestive of tissue damage or illness.

 

The signs and symptoms of HELLP syndrome are similar to those of various other conditions. Clinicians may misdiagnose HELLP syndrome as:

  • Hepatitis. This is liver inflammation.
  • Thrombotic thrombocytopenic purpura (TTP). blood clots are formed in small blood vessels in the body. These clots may block flow of blood to vital body organs causing serious medical conditions
  • Flu or other diseases caused by a virus.
  • Gallbladder disease. The gallbladder makes bile that enables the liver to emulsify fats.
  • Idiopathic thrombocytopenic purpura (ITP). these condition can make a person to bruise easily, and sometimes bleed easily (and excessively).
  • Lupus flare. Lupus is an autoimmune condition. In pregnancy, it can cause medical problems.

 

 Diagnostic Procedures & Tests

How is HELLP syndrome diagnosed?

  •  Review of patient’s history.
  • Performing a physical examination.
  • Tests and other procedures:
  1. Measurement of blood pressure.
  2. Complete blood count with differential including platelets and red blood cells.
  3. Peripheral blood smear (PBF).
  4. Blood tests for enzymes that are indicative of cellular damage.
  5. Measuring bilirubin levels. Bilirubin is a substance that results due to hemolysis of red cells.
  6. Liver function test (LFTs).
  7. Urine tests for protein.

 

Differential Diagnosis

  • Thrombotic thrombocytopenic purpura.
  • Acute fatty liver of pregnancy.
  • Atypical hemolytic uremic syndrome.

 

 

 Treatment and Management

How is HELLP syndrome treated?

Mothers with this condition may need to undergo preterm delivery as soon as possible. The baby is born before 37 weeks of gestation are completed.

Antenatal corticosteroids are given to mothers to help the fetal lungs grow speedily.

Being that this kind of birth is a preterm delivery, there is induction of labour through medications or other ways. Apart from this, a medical procedure referred to as Caesarean delivery may be conducted.

These mothers may also require blood transfusion (red cells, platelets).

 

 Complications

What are the complications of HELLP syndrome?

HELLP syndrome can cause other medical conditions such as:

  •  Placental abruption. The placenta and the wall of the uterus become separated even before birth occurs.
  • Bleeding and blood clotting disorders. Some women who have this syndrome can end up developing a condition called disseminated intravascular coagulopathy (DIC). This disorder of blood clotting can cause hemorrhage.
  • Liver hemorrhage or hepatic failure.
  • Pulmonary edema. This is accumulation of fluid in the lungs and can cause breathing difficulties.
  • Anemia.

 

Prevention

How do we prevent HELLP syndrome?

There are no known methods for preventing this syndrome. However, there are several strategies that can be employed by pregnant women reduce risk factors.

These are:

  • Regularly visiting the clinic (prenatal visits) during pregnancy.
  • Informing your clinicians about any previous hypertensive conditions, preeclampsia and family history of HELLP syndrome.
  • Have conversations about aspirin with your healthcare provider. It has been shown that aspirin can lower the chance of developing preeclampsia, which in turn lowers the risk of developing HELLP syndrome.

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