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Potentially life-threatening emergency
HELLP syndrome
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Potentially life-threatening emergency

HELLP syndrome

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Contributors: Stefanie Hollenbach MD, Mitchell Linder MD
Other Resources UpToDate PubMed

Synopsis

HELLP syndrome is a rare, potentially life-threatening pregnancy complication defined by hemolysis, elevated liver enzymes, and low platelet count. While HELLP syndrome is considered to be a variant of preeclampsia, both proteinuria and hypertension are less predictably present in this condition.

HELLP syndrome shares some risk factors with those of preeclampsia, including chronic hypertension, pregestational renal disease, pregestational diabetes, obesity, and family history of preeclampsia spectrum disorders. However, women with HELLP syndrome are more likely to be of advancing maternal age and/or multiparous than those with preeclampsia without end-organ damage. Complications of pregnancy can also increase the risk of preeclamptic spectrum disorders including molar pregnancies and multifetal pregnancies.

While preeclampsia occurs in approximately 4% of pregnancies, HELLP syndrome occurs in 0.5%-0.9%. However, of the pregnancies affected by severe preeclampsia, 10%-20% will eventually develop HELLP syndrome. Over two-thirds of cases of HELLP syndrome develop in the antepartum period, with nearly all other cases developing within 48 hours following delivery, though it may manifest up to 7 days after delivery.

Clinical presentations are variable. Initial presentations may include any combination of abdominal pain and tenderness (right upper quadrant or epigastric), nausea, vomiting, edema, malaise, headache, and/or visual changes. The initial disease process may also be asymptomatic.

Complications of HELLP syndrome include placental abruption, postpartum hemorrhage, disseminated intravascular coagulation (DIC), pulmonary edema, acute renal failure, and hemorrhagic stroke. Rarely, HELLP-related retinopathy can cause temporary or permanent bilateral visual loss, and liver dysfunction can culminate in spontaneous rupture of a subcapsular liver hematoma. Any of these significant complications can result in maternal and/or fetal death. 

Codes

ICD10CM:
O14.20 – HELLP syndrome (HELLP), unspecified trimester

SNOMEDCT:
95605009 – HELLP Syndrome

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Preeclampsia spectrum disorders – Onset ≥ 20 weeks gestation of new or worsening hypertension and proteinuria with or without lab abnormalities. 
  • Acute fatty liver of pregnancy (see acute liver failure) – Presence of hypoglycemia and prolonged prothrombin time (PT) are the characteristic lab findings in this diagnosis, although there is significant overlap with pure HELLP. 
  • Gestational thrombocytopenia – Typical onset in the second or third trimester of isolated thrombocytopenia with steady (not precipitous) decrease as the end of pregnancy approaches. 
  • Idiopathic thrombocytopenia – Platelet trend tends to be more stable than that of HELLP, onset at any time in pregnancy. Especially consider if low platelets have been noted pregestationally. 
  • Systemic lupus erythematosus – First presentation or flare can look significantly like HELLP. Does not improve with delivery. 
  • Catastrophic antiphospholipid antibody syndrome
  • Thrombotic thrombocytopenic purpura – Neurologic manifestations (confusion, aphasia, weakness) more prominent. 
  • Hemolytic uremic syndrome – More likely to occur postpartum, renal dysfunction is predominant. 
  • Primary gastrointestinal pathologies – Viral hepatitis (eg, A, B, C), cholangitis, cholecystitis, acute pancreatitis

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed: 06/07/2017
Last Updated: 11/20/2017
Copyright © 2019 VisualDx®. All rights reserved.
Potentially life-threatening emergency
HELLP syndrome
Print 1 Images
HELLP syndrome : Nausea, ALT elevated, AST elevated, Easy bruising, Hemolytic anemia, RUQ pain, PLT decreased
Copyright © 2019 VisualDx®. All rights reserved.