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HELLP syndrome

Hemolysis, Elevated Liver function tests, Low Platelets. Epidemiology: - 10% of patients with pre-eclamsia develop HELLP syndrome Pathology: 1) variant of pre-eclampsia 2) microangiopathic hemolytic anemia 3) may be difficult to distinguish from TTP/HUS 4) causes significant maternal & fetal morbidity & mortality 5) hepatic infarctions, hematomas & rupture may occur - liver biopsy shows necrosis, fibrosis, steatosis, hemorrhage 6) fulminant hepatic failure may occur Clinical manifestations: - occurs only occurs during 3rd trimester of pregnancy - features of pre-eclampsia [2] - mild hypertension - right upper quadrant pain, nausea [2] - disseminated intravascular coagulation usually present [2] Laboratory: 1) complete blood count (CBC) with peripheral smear a) microangiopathic hemolytic anemia b) thrombocytopenia 2) liver function tests a) elevated serum aminotransferases - serum ALT (< 500 U/L) - serum AST (< 500 U/L) b) serum: bilirubin: 1] mild hyperbilirubinemia (2-3 mg/dL) 2] elevated serum conjugated bilirubin 3) serum lactate dehydrogenase is elevated 4) renal function tests b) blood urea nitrogen: uremia b) 24 hour urine volume: oliguria c) 24 hour urine protein: proteinuria - 6% of patients without proteinuria [2] 5) prothrombin time (PT) & aPTT are normal 6) plasma D-dimer usually elevated [2] 6) serum glucose may be low 7) see ARUP consult [3] Differential diagnosis: - intrahepatic cholestasis of pregnancy - acute fatty liver of pregnancy - microangiopathic hemolytic distinguishes HELLP syndrome Management: 1) immediate delivery of infant 2) generally resolves 48 hours after delivery (resolution within days to weeks [2])

Related

hemolytic uremic syndrome (HUS) pre-eclampsia/eclampsia thrombotic thrombocytopenic purpura (TTP)

General

liver disease during pregnancy pregnancy-induced hypertension; gestational hypertension (PIH) thrombotic microangiopathy thrombocytopenia syndrome

References

  1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 619
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015 - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  3. ARUP Consult: HELLP Syndrome The Physician's Guide to Laboratory Test Selection & Interpretation https://www.arupconsult.com/content/hellp-syndrome
  4. D'Angelo A, Fattorini A, Crippa L. Thrombotic microangiopathy in pregnancy. Thromb Res. 2009;123 Suppl 2:S56-62 PMID: 19217478