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HELLP syndrome

INTRODUCTION

HELLP is an acronym that refers to a syndrome characterized by Hemolysis with a microangiopathic blood smear, Elevated Liver enzymes, and a Low Platelet count [1]. It probably represents a severe form of preeclampsia (table 1 and table 2), but the relationship between the two disorders remains controversial. As many as 15 to 20 percent of patients with HELLP syndrome do not have antecedent hypertension or proteinuria, leading some authorities to believe that HELLP is a separate disorder from preeclampsia [2-4]. Both severe preeclampsia and HELLP syndrome may be associated with serious hepatic manifestations, including infarction, hemorrhage, and rupture.

This topic will focus upon the clinical manifestations, diagnosis, and management of HELLP syndrome. Preeclampsia is reviewed in detail separately. (See "Preeclampsia: Clinical features and diagnosis" and "Preeclampsia: Management and prognosis".)

INCIDENCE

HELLP develops in approximately 0.1 to 0.8 percent of pregnancies overall and in 10 to 20 percent of women with severe preeclampsia/eclampsia.

RISK FACTORS

A previous history of preeclampsia or HELLP is a risk factor for HELLP syndrome (see 'Recurrence in subsequent pregnancies' below). Sisters and offspring of women with a history of HELLP syndrome are also at increased risk of developing the syndrome [5]. A variety of genetic variants associated with an increased risk of HELLP syndrome have been reported, but have no role in clinical management [6].

In contrast to preeclampsia, nulliparity is not a risk factor for HELLP syndrome [7]. Half or more of affected patients are multiparous.

                            

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Literature review current through: Apr 2013. | This topic last updated: Apr 22, 2013.
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References
Top
  1. Stone JH. HELLP syndrome: hemolysis, elevated liver enzymes, and low platelets. JAMA 1998; 280:559.
  2. Sibai BM, Taslimi MM, el-Nazer A, et al. 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. Reubinoff BE, Schenker JG. HELLP syndrome--a syndrome of hemolysis, elevated liver enzymes and low platelet count--complicating preeclampsia-eclampsia. Int J Gynaecol Obstet 1991; 36:95.
  4. Sibai BM. The HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets): much ado about nothing? Am J Obstet Gynecol 1990; 162:311.
  5. Lachmeijer AM, Arngrímsson R, Bastiaans EJ, et al. A genome-wide scan for preeclampsia in the Netherlands. Eur J Hum Genet 2001; 9:758.
  6. 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; 166:117.
  7. Audibert F, Friedman SA, Frangieh AY, Sibai BM. Clinical utility of strict diagnostic criteria for the HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome. Am J Obstet Gynecol 1996; 175:460.
  8. Benedetto C, Marozio L, Tancredi A, et al. Biochemistry of HELLP syndrome. Adv Clin Chem 2011; 53:85.
  9. Jebbink J, Wolters A, Fernando F, et al. Molecular genetics of preeclampsia and HELLP syndrome - a review. Biochim Biophys Acta 2012; 1822:1960.
  10. Burwick RM, Feinberg BB. Eculizumab for the treatment of preeclampsia/HELLP syndrome. Placenta 2013; 34:201.
  11. Salmon JE, Heuser C, Triebwasser M, et al. Mutations in complement regulatory proteins predispose to preeclampsia: a genetic analysis of the PROMISSE cohort. PLoS Med 2011; 8:e1001013.
  12. Yang Z, Yamada J, Zhao Y, et al. Prospective screening for pediatric mitochondrial trifunctional protein defects in pregnancies complicated by liver disease. JAMA 2002; 288:2163.
  13. Yang Z, Zhao Y, Bennett MJ, et al. Fetal genotypes and pregnancy outcomes in 35 families with mitochondrial trifunctional protein mutations. Am J Obstet Gynecol 2002; 187:715.
  14. Wilcken B, Leung KC, Hammond J, et al. Pregnancy and fetal long-chain 3-hydroxyacyl coenzyme A dehydrogenase deficiency. Lancet 1993; 341:407.
  15. Ibdah JA, Bennett MJ, Rinaldo P, et al. A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women. N Engl J Med 1999; 340:1723.
  16. Sibai BM, Ramadan MK, Usta I, et al. Maternal morbidity and mortality in 442 pregnancies with hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome). Am J Obstet Gynecol 1993; 169:1000.
  17. Catanzarite VA, Steinberg SM, Mosley CA, et al. Severe preeclampsia with fulminant and extreme elevation of aspartate aminotransferase and lactate dehydrogenase levels: high risk for maternal death. Am J Perinatol 1995; 12:310.
  18. Isler CM, Rinehart BK, Terrone DA, et al. Maternal mortality associated with HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome. Am J Obstet Gynecol 1999; 181:924.
  19. Sibai BM. Diagnosis, controversies, and management of the syndrome of hemolysis, elevated liver enzymes, and low platelet count. Obstet Gynecol 2004; 103:981.
  20. Martin JN Jr, Blake PG, Perry KG Jr, et al. The natural history of HELLP syndrome: patterns of disease progression and regression. Am J Obstet Gynecol 1991; 164:1500.
  21. Martin JN Jr, Rinehart BK, May WL, et al. The spectrum of severe preeclampsia: comparative analysis by HELLP (hemolysis, elevated liver enzyme levels, and low platelet count) syndrome classification. Am J Obstet Gynecol 1999; 180:1373.
  22. Page LM, Girling JC. A novel cause for abnormal liver function tests in pregnancy and the puerperium: non-alcoholic fatty liver disease. BJOG 2011; 118:1532.
  23. Pereira SP, O'Donohue J, Wendon J, Williams R. Maternal and perinatal outcome in severe pregnancy-related liver disease. Hepatology 1997; 26:1258.
  24. Innes AM, Seargeant LE, Balachandra K, et al. Hepatic carnitine palmitoyltransferase I deficiency presenting as maternal illness in pregnancy. Pediatr Res 2000; 47:43.
  25. Matern D, Hart P, Murtha AP, et al. Acute fatty liver of pregnancy associated with short-chain acyl-coenzyme A dehydrogenase deficiency. J Pediatr 2001; 138:585.
  26. Sibai BM, Ramadan MK. Acute renal failure in pregnancies complicated by hemolysis, elevated liver enzymes, and low platelets. Am J Obstet Gynecol 1993; 168:1682.
  27. Weiner CP. Thrombotic microangiopathy in pregnancy and the postpartum period. Semin Hematol 1987; 24:119.
  28. Egerman RS, Witlin AG, Friedman SA, Sibai BM. Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome in pregnancy: review of 11 cases. Am J Obstet Gynecol 1996; 175:950.
  29. Sullivan CA, Magann EF, Perry KG Jr, et al. The recurrence risk of the syndrome of hemolysis, elevated liver enzymes, and low platelets (HELLP) in subsequent gestations. Am J Obstet Gynecol 1994; 171:940.
  30. Stella CL, Dacus J, Guzman E, et al. The diagnostic dilemma of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome in the obstetric triage and emergency department: lessons from 4 tertiary hospitals. Am J Obstet Gynecol 2009; 200:381.e1.
  31. Martin JN Jr, Bailey AP, Rehberg JF, et al. Thrombotic thrombocytopenic purpura in 166 pregnancies: 1955-2006. Am J Obstet Gynecol 2008; 199:98.
  32. Visser W, Wallenburg HC. Temporising management of severe pre-eclampsia with and without the HELLP syndrome. Br J Obstet Gynaecol 1995; 102:111.
  33. van Pampus MG, Wolf H, Westenberg SM, et al. Maternal and perinatal outcome after expectant management of the HELLP syndrome compared with pre-eclampsia without HELLP syndrome. Eur J Obstet Gynecol Reprod Biol 1998; 76:31.
  34. O'Brien JM, Shumate SA, Satchwell SL, et al. Maternal benefit of corticosteroid therapy in patients with HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome: impact on the rate of regional anesthesia. Am J Obstet Gynecol 2002; 186:475.
  35. Matchaba P, Moodley J. Corticosteroids for HELLP syndrome in pregnancy. Cochrane Database Syst Rev 2004; :CD002076.
  36. Isler CM, Barrilleaux PS, Magann EF, et al. A prospective, randomized trial comparing the efficacy of dexamethasone and betamethasone for the treatment of antepartum HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome. Am J Obstet Gynecol 2001; 184:1332.
  37. Martin JN Jr, Thigpen BD, Rose CH, et al. Maternal benefit of high-dose intravenous corticosteroid therapy for HELLP syndrome. Am J Obstet Gynecol 2003; 189:830.
  38. Fonseca JE, Méndez F, Cataño C, Arias F. Dexamethasone treatment does not improve the outcome of women with HELLP syndrome: a double-blind, placebo-controlled, randomized clinical trial. Am J Obstet Gynecol 2005; 193:1591.
  39. Katz L, de Amorim MM, Figueiroa JN, Pinto e Silva JL. Postpartum dexamethasone for women with hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome: a double-blind, placebo-controlled, randomized clinical trial. Am J Obstet Gynecol 2008; 198:283.e1.
  40. Woudstra DM, Chandra S, Hofmeyr GJ, Dowswell T. Corticosteroids for HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome in pregnancy. Cochrane Database Syst Rev 2010; :CD008148.
  41. Martin JN Jr, Rose CH, Briery CM. Understanding and managing HELLP syndrome: the integral role of aggressive glucocorticoids for mother and child. Am J Obstet Gynecol 2006; 195:914.
  42. Barton JR, Sibai BM. Hepatic imaging in HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count). Am J Obstet Gynecol 1996; 174:1820.
  43. Nunes JO, Turner MA, Fulcher AS. Abdominal imaging features of HELLP syndrome: a 10-year retrospective review. AJR Am J Roentgenol 2005; 185:1205.
  44. Greenstein D, Henderson JM, Boyer TD. Liver hemorrhage: recurrent episodes during pregnancy complicated by preeclampsia. Gastroenterology 1994; 106:1668.
  45. Wicke C, Pereira PL, Neeser E, et al. Subcapsular liver hematoma in HELLP syndrome: Evaluation of diagnostic and therapeutic options--a unicenter study. Am J Obstet Gynecol 2004; 190:106.
  46. Schwartz ML, Lien JM. Spontaneous liver hematoma in pregnancy not clearly associated with preeclampsia: a case presentation and literature review. Am J Obstet Gynecol 1997; 176:1328.
  47. Rinehart BK, Terrone DA, Magann EF, et al. Preeclampsia-associated hepatic hemorrhage and rupture: mode of management related to maternal and perinatal outcome. Obstet Gynecol Surv 1999; 54:196.
  48. Grand'Maison S, Sauvé N, Weber F, et al. Hepatic rupture in hemolysis, elevated liver enzymes, low platelets syndrome. Obstet Gynecol 2012; 119:617.
  49. Wilson RH, Marshall BM. Postpartum rupture of a subcapsular hematoma of the liver. Acta Obstet Gynecol Scand 1992; 71:394.
  50. Stevenson JT, Graham DJ. Hepatic hemorrhage and the HELLP syndrome: a surgeon's perspective. Am Surg 1995; 61:756.
  51. Merchant SH, Mathew P, Vanderjagt TJ, et al. Recombinant factor VIIa in management of spontaneous subcapsular liver hematoma associated with pregnancy. Obstet Gynecol 2004; 103:1055.
  52. Erhard J, Lange R, Niebel W, et al. Acute liver necrosis in the HELLP syndrome: successful outcome after orthotopic liver transplantation. A case report. Transpl Int 1993; 6:179.
  53. Hunter SK, Martin M, Benda JA, Zlatnik FJ. Liver transplant after massive spontaneous hepatic rupture in pregnancy complicated by preeclampsia. Obstet Gynecol 1995; 85:819.
  54. Araujo AC, Leao MD, Nobrega MH, et al. Characteristics and treatment of hepatic rupture caused by HELLP syndrome. Am J Obstet Gynecol 2006; 195:129.
  55. Zarrinpar A, Farmer DG, Ghobrial RM, et al. Liver transplantation for HELLP syndrome. Am Surg 2007; 73:1013.
  56. Ilbery M, Jones AR, Sampson J. Lupus anticoagulant and HELLP syndrome complicated by placental abruption, hepatic, dermal and adrenal infarction. Aust N Z J Obstet Gynaecol 1995; 35:215.
  57. Alsulyman OM, Castro MA, Zuckerman E, et al. Preeclampsia and liver infarction in early pregnancy associated with the antiphospholipid syndrome. Obstet Gynecol 1996; 88:644.
  58. Seige M, Schweigart U, Moessmer G, et al. Extensive hepatic infarction caused by thrombosis of right portal vein branches and arterial vasospasm in HELLP syndrome associated with homozygous factor V Leiden. Am J Gastroenterol 1998; 93:473.
  59. Pauzner R, Dulitzky M, Carp H, et al. Hepatic infarctions during pregnancy are associated with the antiphospholipid syndrome and in addition with complete or incomplete HELLP syndrome. J Thromb Haemost 2003; 1:1758.
  60. Chou MM, Chen YF, Kung HF, et al. Extensive hepatic infarction in severe preeclampsia as part of the HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets): evolution of CT findings and successful treatment with plasma exchange therapy. Taiwan J Obstet Gynecol 2012; 51:418.
  61. Hupuczi P, Nagy B, Sziller I, et al. Characteristic laboratory changes in pregnancies complicated by HELLP syndrome. Hypertens Pregnancy 2007; 26:389.
  62. Martin JN Jr, Blake PG, Lowry SL, et al. Pregnancy complicated by preeclampsia-eclampsia with the syndrome of hemolysis, elevated liver enzymes, and low platelet count: how rapid is postpartum recovery? Obstet Gynecol 1990; 76:737.
  63. Knapen MF, van Altena AM, Peters WH, et al. Liver function following pregnancy complicated by the HELLP syndrome. Br J Obstet Gynaecol 1998; 105:1208.
  64. Jacquemyn Y, Jochems L, Duiker E, et al. Long-term renal function after HELLP syndrome. Gynecol Obstet Invest 2004; 57:117.
  65. Drakeley AJ, Le Roux PA, Anthony J, Penny J. Acute renal failure complicating severe preeclampsia requiring admission to an obstetric intensive care unit. Am J Obstet Gynecol 2002; 186:253.
  66. Sibai BM, Ramadan MK, Chari RS, Friedman SA. Pregnancies complicated by HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets): subsequent pregnancy outcome and long-term prognosis. Am J Obstet Gynecol 1995; 172:125.
  67. Chames MC, Haddad B, Barton JR, et al. Subsequent pregnancy outcome in women with a history of HELLP syndrome at < or = 28 weeks of gestation. Am J Obstet Gynecol 2003; 188:1504.
  68. van Pampus MG, Wolf H, Mayruhu G, et al. Long-term follow-up in patients with a history of (H)ELLP syndrome. Hypertens Pregnancy 2001; 20:15.
  69. Habli M, Eftekhari N, Wiebracht E, et al. Long-term maternal and subsequent pregnancy outcomes 5 years after hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome. Am J Obstet Gynecol 2009; 201:385.e1.
  70. Wüst MD, Bolte AC, de Vries JI, et al. Pregnancy outcome after previous pregnancy complicated by hepatic rupture. Hypertens Pregnancy 2004; 23:29.
  71. Abramovici D, Friedman SA, Mercer BM, et al. Neonatal outcome in severe preeclampsia at 24 to 36 weeks' gestation: does the HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome matter? Am J Obstet Gynecol 1999; 180:221.
  72. Sibai BM, Spinnato JA, Watson DL, et al. Pregnancy outcome in 303 cases with severe preeclampsia. Obstet Gynecol 1984; 64:319.
  73. Harms K, Rath W, Herting E, Kuhn W. Maternal hemolysis, elevated liver enzymes, low platelet count, and neonatal outcome. Am J Perinatol 1995; 12:1.
  74. Singhal N, Amin HJ, Pollard JK, et al. Maternal haemolysis, elevated liver enzymes and low platelets syndrome: perinatal and neurodevelopmental neonatal outcomes for infants weighing less than 1250 g. J Paediatr Child Health 2004; 40:121.
  75. Dötsch J, Hohmann M, Kühl PG. Neonatal morbidity and mortality associated with maternal haemolysis elevated liver enzymes and low platelets syndrome. Eur J Pediatr 1997; 156:389.
  76. Gortner L, Pohlandt F, Bartmann P, et al. Short-term outcome in infants with birth weights less than 1750 g born to mothers with HELLP syndrome. J Perinat Med 1992; 20:25.
  77. Kändler C, Kevekordes B, Zenker M, et al. Prognosis of children born to mothers with HELLP-syndrome. J Perinat Med 1998; 26:486.
  78. Murray D, O'Riordan M, Geary M, et al. The HELLP syndrome: maternal and perinatal outcome. Ir Med J 2001; 94:16.
  79. Guzel AI, Kuyumcuoglu U, Celik Y. Are maternal and fetal parameters related to perinatal mortality in HELLP syndrome? Arch Gynecol Obstet 2011; 283:1227.