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1999-09-27-09 HELLP syndrome © Silva www.thefetus.net/

HELLP syndrome

Sandra Silva, MD, Philippe Jeanty, MD, PhD

Definition: HELLP syndrome is an acronym for a severe variant of preeclampsia, characterized by hemolysis, elevated liver enzymes, and low platelets[1]. This condition is life threatening for both mother and fetus. Sonographic signs of fetal compromise include IUGR, decreased tonus, decreased movement, and abnormal Doppler of the umbilical and cerebral arteries.

Incidence: HELLP syndrome affects 2% to 12% of the patients who develop preeclampsia[2], and Caucasian women seem to be more affected than African-American women[3].

Etiology:  HELLP syndrome is a situation specific of pregnancies complicated by severe preeclampsia.

Recurrence risk: Although the risk of recurrence is not precisely known, every patient affected by HELLP syndrome should be followed in a subsequent pregnancy as a patient at increased risk[4].

Diagnosis: The maternal diagnosis is made by laboratory studies (hemolysis, elevated liver enzymes, and thrombocytopenia – platelets bellow 100,000, which is the most consistent finding) and clinical signs and symptoms (which includes edema, hypertension, nausea, and abdominal pain, due to hepatic hemorrhage and/or rupture). The fetal findings include IUGR, and reduced amniotic fluid volume, which are fetal responses to the chronic process of decreased placental blood supply, resulting from the maternal hypertension. In a more severe situation, with diminished fetal oxygen reserve, signs of distress may be found, such as decreased tonus, movement, respiratory movement, and abnormal Doppler velocimetry. Fetal demise and neonatal death are not uncommon in severe cases.

Associated anomalies: A very important and dangerous condition that may associates with HELLP syndrome is disseminated intravascular coagulation (DIC)[5].

Differential diagnosis: Disorders that can develop liver dysfunction or anemia, such as thrombocytopenic purpura, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, gallbladder disease, viral hepatitis, and acute fatty liver of pregnancy. 

Prognosis: The prognosis is variable, according to the severity of both maternal and fetal status. Maternal morbidity and mortality are proportionate to the severity of systemic disease, whereas fetal morbidity and mortality are gestational-age dependent in the majority of cases2.

Management: Patients with HELLP should be considered as having severe preeclampsia. Referral to a tertiary center as well as delivery of term pregnancies is recommended. Cases remote from term of pregnancy should be managed conservatively, with intensive monitoring of both maternal and fetal well being. Delivery should be accomplished as soon as pulmonary maturity is reached. 

References:


[1] Weinstein L. Syndrome of hemolysis, elevated liver enzymes, and low platelet count: a severe consequence of hypertension in pregnancy. Am J Obstet Gynecol 1982, 142:159.

[2] Sibai BM, Taslimi MM, El-Nazer A, Amon E, Mabie WC, Ryan G. Maternal perinatal outcome associated with the syndrome of hemolysis, elevated liver enzymes, and low platelets in severe preeclampsia-eclampsia. Am J Obstet Gynecol 1986, 155: 501.

[3] Goodlin RC. Beware the great imitator-severe preeclampsia. Contemp Obstet Gynecol 1984, 20:215.

[4] Fairlie FM, Sibai BM. Hypertensive diseases in pregnancy In Medicine of the Fetus & Mother from Reece EA, Hobbins JC, Mahoney MJ, Petrie RH. J.B. Lippincott Company –Philadelphia 1992, 58: 925-942.

[5] Van Dam PA, Renier M, Baekelandt M, Buytaert P, Uyttenbroeck F. Disseminated intravascular coagualtion and the syndrome of hemolysis, elevated liver enzymes, and low platelets in severe preeclampsia. Obstet Gynecol 1989, 73:97.

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