HELLP syndrome is a life threatening variant of preeclampsia, characterized by a group of serious symptoms and complications that generally occur late in pregnancy or after delivery. Dr. Louis Weinstein named the condition using the initials of its main features , with the full form being:
It is classified into three categories  using the Mississippi classification system which determines the severity of the condition depending on the reduction in the patient’s blood platelet count.
Researchers are still trying to find out the exact causes responsible for this pregnancy related problem. However, certain risk factors have been identified by doctors .
Although the triggering factors are still unknown, experts believe the condition to occur when the coagulation cascade gets activated due to abnormal functioning of the body. Fibrin creates conjoined networks within the small blood vessels, leading to microangiopathic hemolytic anemia. The mesh formed by the fibrin destroys red blood cells as they pass through it. Considerable numbers of platelets are destroyed as well. As the whole process takes place in the liver, the downstream liver cells develop ischemia which leads to periportal necrosis. The disorder may equally affect cells in various other organs. In some cases, it may become quite difficult to perform an emergency surgery as HELLP syndrome gradually leads to a certain form of DIC (disseminated intravascular coagulation), resulting in paradoxical bleeding.
During the initial stages, the patient may or may not have the symptoms of high blood pressure, proteinuria (protein in the urine) and high uric acid levels in urine. These are the characteristic symptoms of preeclampsia. The onset of the symptoms occurs during the second or third trimester. Women suffering from HELLP syndrome often experience:
Although it is not possible to prevent the condition from occurring, taking the following measures may reduce the risks :
The diagnostician should look for the laboratory criteria  mentioned below to confirm the diagnosis:
1) Hemolysis, accompanied by burr cells, polychromasia and schistocytes shown on a peripheral blood smear test. However, peripheral smears are not always used for this diagnosis as the presence of Hemolysis can be confirmed from the following evidence:
2) Elevated liver transaminases: Aspartate aminotransferase (AST) and/or alanine transaminase (ALT) >70 IU/L
3) Diagnostic tests used for confirming the presence of HELLP Syndrome include:
Radiology and CT scan may be necessary in case of any possible complications.
Sometimes, the absence of the primary preeclampsia symptoms may lead the diagnostician to mistake the condition for some other disorder . The initial symptoms of HELLP Syndrome are similar to those caused by:
The treatment mainly focuses on management of the symptoms and prevention of any serious complication. Delivering the baby is the most effective way to prevent the disorder from leading to any complication in the mother or the baby.
In cases where the HELLP syndrome occurs within 34 weeks of gestation, the baby’s lung functioning is evaluated by doctors for determining the possible effects of delivery on the baby. Treatment guidelines for managing the condition until the baby is mature enough for delivery usually include:
Drugs and medications used for the purpose include
Postpartum HELLP syndrome treatment may involve administration of heparin shots.
The symptoms of HELLP Syndrome tend to worsen postpartum before resolving entirely (within 2 to 3 days) with proper treatment and medications. The time required to reach complete recovery may vary in cases where complications are present.
Maternal mortality rate is as low as 1% with proper treatment. The number of prenatal and infant death ranges from 73 to 119 per 1000 cases. Around 40% babies born to HELLP affected mothers are delivered prematurely, hence are smaller than the ideal birth size. The outcome and long term effects of HELLP in the baby are determined by factors like the gestational age rather than the severity of the disorder itself.
The recurrence rate ranges between 2% and 19% depending on whether one suffered from preeclampsia in an earlier pregnancy.
The ICD-10 Code for HELLP syndrome is O14.2.
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