HELLP Syndrome

Definition

HELLP Syndrome is considered to be a life-threatening complication of pregnancy. It is commonly contemplated to be a form of preeclampsia which is a condition developed by pregnant women and is distinguished by certain signs of damage on another organ system and high blood pressure. HELLP syndrome is a very dangerous condition that exists in pregnant women who have the following:

• H: hemolysis
• EL: elevated liver enzymes
• LP: low platelet count

Hemolysis is defined as the breakdown of red blood cells. Particularly, the red blood cells are broken down earlier than usual. Hemolysis could result to anemia which is another condition in which the blood does not contain enough amount of oxygen to supply the body.

Elevated liver enzymes signify that the liver is not functioning properly. When the liver cells are injured or swollen, they secrete an excessive amount of certain chemicals, which include enzymes, directly into the blood.

Platelets are tiny pieces of blood cells that help prevent bleeding and heal wounds by forming blood clots. When the platelets are low, an individual is at risk for excessive bleeding.

HELLP syndrome typically appears in the last trimester of the pregnancy, specifically before the 37th week. The concern is very significant since it could be lethal to both the unborn baby and the mother. Immediate treatment and delivery of the baby are the best option that is required for an excellent outcome. However, there is a third reported case of HELLP syndrome that occurred after the baby is successfully born in the first week after the delivery. In accordance to the National Institutes of Health, HELLP syndrome is estimated to affect one to two in every 1,000 pregnant women.

Symptoms of HELLP syndrome

Signs and symptoms of this problem are much the same with flu-like symptoms. Some symptoms of this condition may seem to be the normal pregnancy side-effects when in fact it is not. Sadly, a lot of women do not seek for any medical treatments because they misdiagnose themselves. It is essential to remember that if any flu-like symptoms happen to occur during pregnancy, have it assessed by a healthcare professional to certify that they are not indicating serious health problems.

Pregnant women experience one or more of the following symptoms:

• Headache
• Nausea
• Indigestion that is accompanied by pain after eating
• Vomiting
• Inflammation, especially on the face or hands
• Unwell feeling or fatigued
• Abdominal pain, particularly in the upper-right side of the abdomen just below the ribs
• Visual disturbances
• Shoulder pain
• Excessively gaining weight
• Heartburn
• Shoulder pain

Other signs to look for:

• Presence of protein in the urine
• High blood pressure

In rare cases, these may also occur:

• Seizures
• Nosebleeds or other kind of bleeding that is difficult to control

The most common reasons that are believed to be the cause for mothers, who become dangerously ill or may die, are stroke or liver rupture. These could still be prevented if caught in time.

Pathophysiology

The exact cause of HELLP syndrome is still unknown. However, some experts theorize that it’s somehow related to preeclampsia, the pathophysiology stems from a typical source. The general activation of the coagulation cascade is deemed to be the primary basis of the problem.

Fibrin creates cross lined networks inside the small blood vessels that result to a microangiopathic hemolytic anemia; the barrier leads to the damaging of red blood cells, like they were forced through a strainer. In addition to that, platelets are also ingested.

The liver is the site where the process occurs that will later result to periportal necrosis. Other certain organs can be affected as well. HELLP syndrome could be the reason of another variant form of disseminated intravascular coagulation (DIC), resulting to paradoxical bleeding, which will serve as a difficult task to perform an emergency surgery.

Other theories involve congenital errors of fatty acid oxidative metabolism coming after a long and medium chain fatty mutation of acid, which can cause liver damage secondary to inadequate mitochondrial oxidation of fatty acids needed for ketogenesis. Another proposed theory of a placental-instigated acute inflammatory disease targets the liver.

Impairment in the function of the complement system from faulty regulation or immoderate activation of endothelial injury has also been suggested to cause certain damage to hepatic vessels in HELLP syndrome.

A lot of researchers make hypotheses in attempt to define and explain the pathogenesis of the condition, but the true pathology is still a puzzle.

Classification

HELLP syndrome is classified into three categories based utilizing “the Mississippi classification” in which the severity of the condition is determined depending on the blood platelet count. These are:

1. Class I – Severe thrombocytopenia with platelets below 50,000/mm3
2. Class II – Moderate thrombocytopenia with platelets from 50,000 and 100,000/mm3
3. Class III – Mild thrombocytopenia with platelets between 100,000 and 150, 000/mm3

Diagnosis

HELLP syndrome requires proper medical diagnosis. Possible tests may include:

• Blood tests – To help determine the levels of platelet and red blood cell count
• Urine tests – To identify high liver enzymes
• Computed tomography (CT) scan – An imaging test that could detect bleeding in the liver
• Physical examination – Abdominal tenderness, leg inflammation, high blood pressure, and an enlarged liver can be discovered in this test

The readings of blood pressure might be frequently high with patients who have HELLP syndrome. Protein in the urine may also be present. The physician has to evaluate this at each prenatal visit.

Treatment

The primary treatment for HELLP syndrome is to deliver the baby as early as possible. Problems in the liver and other complications of the condition could rapidly get worse and may harm both the child and mother. The healthcare provider might give medicines to begin with labor, or perform a caesarean section.

The patient may also receive:

• Medications for high blood pressure
• Blood transfusion if there is severe bleeding problems
• Infusion of magnesium sulfate to prevent seizures
• Corticosteroid medicines to help the baby’s lungs mature faster if in case an early delivery is required

References:

1. What Is the Long-Term Outlook for Women with HELLP Syndrome? at http://www.healthline.com/health/hellp-syndrome#Overview1
2. What Can I Do to Prevent HELLP Syndrome?, How HELLP Syndrome Affects Babies at http://www.preeclampsia.org/health-information/hellp-syndrome
3. Hellp Syndrome – Causes, Symptoms, tests, Treatment, Prognosis at https://www.nlm.nih.gov/medlineplus/ency/article/000890.htm
4. Martin JN, Owens MY, Keiser SD, Parrish MR, Tam Tam KB, Brewer JM, Cushman JL, May WL (2012). “Standardized Mississippi Protocol treatment of 190 patients with HELLP syndrome: slowing disease progression and preventing new major maternal morbidity”. Hypertens Pregnancy 31 (1): 79–90.
5. Martin JN Jr, Rose CH, Briery CM (Oct 2006). “Understanding and managing HELLP syndrome: the integral role of aggressive glucocorticoids for mother and child”. Am J Obstet Gynecol 195 (4): 914–34.
6. Weinstein L (Sep 2005). “It has been a great ride: the history of HELLP syndrome”. Am J Obstet Gynecol 193 (3 Pt 1): 860–3.