HELLP syndrome is a pregnancy-related condition and is an abbreviation for:
- elevated liver enzymes and
- low platelets
It is considered a severe and life-threatening form of pre-eclampsia although it can occur without co-existing pre-eclampsia.
The estimated incidence is ~0.5% (range 0.17-0.85%) of live births 9. The condition often occurs in the 3rd trimester of pregnancy (and occasionally postpartum). It tends to present in young primigravid women.
The presentation can be variable and can include malaise, epigastric/right upper-quadrant pain, and nausea/vomiting. Some may have non-specific viral-like symptoms. Hypertension and proteinuria (classic symptoms of preeclampsia) may be absent or slight 7.
The etiology and pathophysiology remain incompletely understood with multiple theories 12:
- immune-mediated: maternal acute rejection reaction to fetal antigens
- placenta-mediated liver injury
- systemic inflammatory response syndrome in the setting of pre-eclampsia
On liver histology, there is may a combination of deposited fibrin, hemorrhage and hepatocellular necrosis surrounding portal areas 6.
- disseminated intravascular coagulation: reported to occur in ~30% (range 20-40%) of patients 5
- hepatic infarction
- hepatic hematoma
- hepatic rupture
- placental abruption
The place of CT is mainly to assess for complications. When considering a CT scan the radiologist needs to discuss with the obstetrician regarding radiation risk to fetus from radiation, versus clinical suspicion of complications in order to make a rational judgment. The CT scan may show hepatic complications such as hemorrhage, rupture, subcapsular hematoma or hepatic infarction.
Treatment and prognosis
Management is often supportive. Patients with hepatic rupture +/- intraperitoneal bleeding require immediate surgery or selective hepatic arterial embolization.
History and etymology
The condition was originally described Pritchard et al. in 1954 3 with the acronym later coined by L Weinstein in 1982 4.
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- 2. Woodfield CA, Lazarus E, Chen KC et-al. Abdominal pain in pregnancy: diagnoses and imaging unique to pregnancy--self-assessment module. AJR Am J Roentgenol. 2010;194 (6): S42-5. doi:10.2214/AJR.10.7224 - Pubmed citation
- 3. Pritchard JA, Weisman R, Ratnoff OD, Vosburgh GJ. Intravascular hemolysis, thrombocytopenia and other hematologic abnormalities associated with severe toxemia of pregnancy. N Engl J Med1954; 250:89 –98
- 4. 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 (2): 159-67. - Pubmed citation
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- 7. Sibai BM. The HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets): much ado about nothing? Am. J. Obstet. Gynecol. 1990;162 (2): 311-6. - Pubmed citation
- 8. Rath W, Faridi A, Dudenhausen JW. HELLP syndrome. J Perinat Med. 2000;28 (4): 249-60. doi:10.1515/JPM.2000.033 - Pubmed citation
- 9. Furlan A, Fakhran S, Federle MP. Spontaneous abdominal hemorrhage: causes, CT findings, and clinical implications. AJR Am J Roentgenol. 2009;193 (4): 1077-87. doi:10.2214/AJR.08.2231 - Pubmed citation
- 10. Haddad B, Barton JR, Livingston JC et-al. HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome versus severe preeclampsia: onset at < or =28.0 weeks' gestation. Am. J. Obstet. Gynecol. 2000;183 (6): 1475-9. Am. J. Obstet. Gynecol. (link) - Pubmed citation
- 11. Vigil-de gracia P. Acute fatty liver and HELLP syndrome: two distinct pregnancy disorders. Int J Gynaecol Obstet. 2001;73 (3): 215-20. Int J Gynaecol Obstet (link) - Pubmed citation
- 12. Abildgaard U, Heimdal K. Pathogenesis of the syndrome of hemolysis, elevated liver enzymes, and low platelet count (HELLP): a review. Eur. J. Obstet. Gynecol. Reprod. Biol. 2013;166 (2): 117-23. doi:10.1016/j.ejogrb.2012.09.026 - Pubmed citation