HELLP syndrome
HELLP syndrome is a life-threatening complication of pre-eclampsia. Both conditions occur during the latter stages of pregnancy, or sometimes after childbirth.
HELLP is an abbreviation of the main findings:
Signs and symptoms
Often, a patient who develops HELLP syndrome has already been followed up for gestational hypertension, or is suspected to develop pre-eclampsia (high blood pressure and proteinuria). Arterial hypertension is a diagnostic requirement. There is gradual but marked onset of headaches, blurred vision, "band pain" around the upper abdomen and tingling in the extremities. If the patient gets a seizure or coma, the condition has progressed into full-blown eclampsia.
Diagnosis
In a patient with possible HELLP syndrome, a batch of blood tests is performed: a full blood count, liver enzymes, renal function and electrolytes and coagulation studies. Often, fibrin degradation products (FDPs) are determined, which can be elevated.
Pathophysiology
The exact cause of HELLP is unknown, but general activation of the coagulation cascade is considered the main underlying problem. Fibrin forms crosslinked networks in the small blood vessels. This leads to a microangiopathic hemolytic anemia: the mesh causes destruction of red blood cells. Additionally, platelets are consumed. As the liver appears to be the main site of this process, downstream liver cells suffer ischemia. Other organs can be similarly affected. HELLP syndrome leads to a variant form of disseminated intravascular coagulation (DIC), leading to paradoxical bleeding, which can make emergency surgery a serious challenge. The liver can rupture as part of the disease process.
Treatment
The only effective treatment is delivery of the baby, preferably by cesarean section. Several medications have been investigated for the treatment of HELLP syndrome, but evidence is conflicting as to whether magnesium sulfate decreases the risk of seizures and progress to eclampsia. The DIC is treated with fresh frozen plasma to replenish the coagulation proteins, and the anemia may require blood transfusion.
