HELLP Syndrome

At a glance

It is an acronym that stands for Hemolysis, Elevated Liver enzymes, Low Platelet counts. This is a life-threatening medical condition with a global mortality reported as high as 25%. Liver rupture, infarction, hemorrhage as well as cerebral edema, hemorrhage, and stroke are often observed in critically ill mothers and after death. It is often considered a variant of preeclampsia. As with preeclampsia, it usually occurs during the later stages of pregnancy, or sometimes after childbirth. It is clinical characterized by gradual but marked onset of headaches (30%), blurred vision, paresthesia, and hypertension (which can be mild). HELLP Syndrome is often difficult to diagnose especially when high blood pressure and protein in the urine are not present. Often, the diagnosis is erroneous as it is mistaken for gastritis, flu, acute hepatitis, gall bladder disease, or other acute ailments.

Synonyms

Hemolytic Liver Platelet Syndrome of Pregnancy.

History

This medical condition was identified as a clinically different entity than severe preeclampsia by Dr Louis Weinstein in 1982. After review of the literature in 2005 and the report of the unexplained postpartum death of a Caucasian woman who presented with severe hemolysis, abnormal liver functions, thrombocytopenia, and marked hypoglycemia, he noticed that this clinical presentation was observed as early as 1954.

Incidence

The true incidence remains unknown in the general population as it is often misdiagnosed for preeclampsia or other medical conditions associated with pregnancies. However, it is believed around 0.5 to 0.9% of all pregnancies. Also, it is associated in 10 to 20% of women with severe preeclampsia. It has been reported that HELLP usually occurs in Caucasian women over the age of 25.

Genetic inheritance

There is no evidence of a genetic association with this medical condition. However, it has been suggested that a possible association exist between an inherited, autosomal recessive abnormality of fatty-acid oxidation caused by a deficiency in the long chain 3-hydroxyacyl-coenzyme dehydrogenase (LCHAD) in the child and the mother HELLP. About 80% of infants with LCHAD deficiency have been born after pregnancies complicated by acute fatty liver of pregnancy (AFLP) or HELLP. Unfortunately, the incidence of pregnancies complicated by AFLP or HELLP as a result in infants with LCHAD deficiency remains unknown.

Pathophysiology

The exact cause of HELLP remains unknown; however, suspicion in favor of a general activation of the coagulation cascade retains most attention. Microangiopathic hemolytic anemia due to activation of fibrin formation in small blood vessels activates this response. Periportal necrosis caused by liver ischemia may lead to liver hemorrhage and rupture. Disseminated intravascular coagulation is activated leading to severe paradoxical bleeding.

Diagnosis

HELLP Syndrome is often difficult to diagnose because of ...