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HELLP syndrome is a rare complication of pregnancy. It is related to preeclampsia, as it typically includes maternal hypertension (high blood pressure) and dysfunction of an organ and is thought to be triggered in some way by the placenta. But there is controversy over whether HELLP is a complication of preeclampsia, a subtype of preeclampsia, or an entity separate from preeclampsia. HELLP is an acronym that stands for Hemolysis, Elevated Liver enzymes, and Low Platelets. Hypertension (high blood pressure) is not included in the acronym, because blood pressure may or may not be elevated early during HELLP, although it typically develops at some point, which is why many experts consider HELLP to be a preeclampsia category. Since there is a lot of medical terminology in the name of the condition, let’s go through what each of the terms means before continuing our discussion of HELLP syndrome.
Hemolysis is the breakage of red blood cells, the cells that carry oxygen and are the most abundant cells in the blood. The liver is an organ that has many different jobs, so its cells, which are called hepatocytes, make a variety of enzymes. Certain liver enzymes tend to leak from the liver into the blood when something is wrong with the liver. The elevation of a handful of liver enzymes in blood is what the EL part of HELLP means. As for platelets, these are cells —or technically cell fragments, since they come from certain cells that break apart— whose job is to stop bleeding when a blood vessel is injured. They do this by attaching together and to the injured part of the blood vessel wall. To have adequate clotting through the body, the concentration of platelets in the blood needs to be above a certain level; otherwise, the person can easily suffer hemorrhage. LP, meaning low platelets, means that the concentration of platelets —the number of platelets per given volume of blood— drops too low. This is called thrombocytopenia and when it happens during pregnancy, along with hemolysis, and a rise in liver enzymes, it’s HELLP syndrome.
Symptoms of HELLP may include severe headache, abdominal pain (especially in the right upper quadrant), nausea, vomiting, and jaundice, which is yellowing of the skin, the sclera of the eyes, and the mucous membranes. You may be thinking that nausea is a normal part of pregnancy, but the usual nausea and vomiting of pregnancy subsides during the early part of the second trimester, generally by around gestational week 16. But HELLP typically begins around the early to mid third trimester and more rarely can develop in the days after delivery.
HELLP syndrome can lead to serious complications for both the mother and the baby. These complications include liver damage, bleeding disorders, and death. In cases of preeclampsia, the only cure is to delivery of the baby and the placenta as soon as possible. The same is true in cases of HELLP syndrome, whether the patient starts out looking as if she has preeclampsia, which then evolves into HELLP, or whether the condition manifests as HELLP syndrome from the beginning.
HELLP syndrome develops in approximately 0.1 percent to 0.6 of pregnancies overall and in 4-12 percent of those already suffering from preeclampsia. These numbers tell you that many cases of HELLP happen in women who do not have preeclampsia, unless you are among those who consider HELLP to be a type of preeclampsia.
In contrast with preeclampsia, which classically strikes the extremes of the age spectrum of expectant mothers —the young (pregnant teens) and the “old” (pregnant women above the age of 35 years— HELLP syndrome tends to strike in the middle of the age spectrum (mid to late 20s). Whereas African American women are particularly vulnerable to develop preeclampsia, HELLP syndrome tends to strike women who are Caucasian and of Asian groups, such as Chinese.
Women suffering from HELLP syndrome are given magnesium sulfate, as a prophylactic treatment to prevent seizures. They also are given corticosteroid treatment, such as dexamethasone, to accelerate the maturation of the fetal lungs. The lung maturation has a lot to do with an ability to produce adequate amounts of surfactant and the appropriate amounts of different types of surfactants. Essentially, surfactant is the soapy substance that is vital, because it enables the lungs to expand easily. Surfactant contains chemicals called phospholipids and the various types of phospholipids must be present in certain ratios. Enabling the lungs to mature is important, because early delivery of the baby and the placenta will terminate the process underlying the HELLP syndrome. In rare cases when HELLP syndrome develops after delivery, things can get quite a bit more complicated.