HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome

Gonca Yenicesu; İclal Kol; Cem Yenicesu; Ali Çetin

EN TR

HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome

Abstract

The HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome is a severe and life-threatening complication of preeclampsia with typical laboratory findings. The frequency of the disease is 1 to 150-300 live births in perinatal centers. The course of the HELLP syndrome is unpredictable. On the one hand, complete reversal of symptoms under conservative treatment have been reported in individual cases, on the other hand, rapid, therapy-resistant deterioration of the disease had been observed in the majority of patients accompanied by severe complications as liver rupture. As a consequence; the mother and the newborn need intensive care and these women should be delivered in an obstetric intensive care unit. The maternal mortality reported from the international literature is 3.3% and the perinatal mortality 22.6%. This review will emphasize the controversies surrounding the diagnosis and management of HELLP syndrome.

Keywords

HELLP (hemoliz, yükselmiş karaciğer enzimleri ve düşük trombosit) sendromu

Öz

HELLP (hemoliz, yükselmiş karaciğer enzimleri ve düşük trombosit) sendromu tipik laboratuar bulguları olan, şiddetli ve hayatı tehdit eden bir preeklampsi komplikasyonudur. Perinatal merkezlerde hastalığın sıklığı 150-300 canlı doğumda 1'dir. HELLP sendromunun seyri tahmin edilemez. Bir tarafta tedavi ile semptomların tam olarak geri döndüğü az sayıdaki vakalar bildirilirken, diğer tarafta hastaların çoğunda karaciğer rüptürü gibi şiddetli komplikasyonların eşlik ettiği, hızlı ve tedaviye dirençli bir kötüye gidiş gözlenmiştir. Sonuç olarak anne ve yenidoğanın yoğun bakıma ihtiyacı vardır ve bu kadınlar bir obstetrik yoğun bakım ünitesinde doğum yapmalıdır. Uluslararası literatürde maternal mortalite %3,3 ve perinatal mortalite %22,6 olarak bildirilmiştir. Bu derleme HELLP sendromunun tanı ve tedavisi ile ilgili tarışmaların üzerinde duracaktır.

Anahtar Kelimeler

Kaynakça

Goodlin RC. Beware the great imitator-severe preeclampsia. Contemp Obstet Gynecol 1984; 20:215.
Weinstein L. Syndrome of haemolysis, elevated liver enzymes and low platelet count; a severe consequence of hypertension in pregnancy. Am J Obstet Gynecol 1982; 142: 159.
Roberts JM, Cooper DW. Pathogenesis and genetics of pre-eclampsia. Lancet 2001; 357: 53-6.
Barton JR, Riely CA, Adamec TA, Shanklin DR, Khoury AD, Sibai BM. Hepatic histopathologic condition does not correlate with laboratory abnormalities in HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count). Am J Obstet Gynecol 1992; 167: 1538-43.
Sibai BM, Ramadan MK, Usta I, Salama M, Mercer BM, Friedman SA.Maternal morbidity and mortality in 442 pregnancies with hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome) Am J Obstet Gynecol 1993; 169: 1000-6.
Santema JG, Koppelaar I, Wallenburg HC. Hypertensive disorders in twin pregnancy. Eur J Obstet Gynecol Reprod Biol 1995; 58: 9-13.
Sibai BM, Taslimi MM, el-Nazer A, Amon E, Mabie BC, Ryan GM. Maternal-perinatal outcome associated with the syndrome of haemolysis, elevated liver enzymes and low platelets in severe preeclampsia-eclampsia. Am J Obstet Gynecol 1986; 155: 501.
Haram K, Svendsen E, Abildgaard U. The HELLP syndrome: clinical issues and management. BMC Pregnancy Childbirth. 2009; 26; 9: 8.

Sibai BM. Diagnosis, controversies, and management of the syndrome of hemolysis, elevated liver enzymes, and low platelet count. Obstet Gynecol 2004; 103: 981-91.
Sibai BM: Imitators of severe pre-eclampsia/eclampsia. Clin Perinatol 2004; 31: 835-52.
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: 914-34.
Martin JN Jr, Rinehart BK, May WL, Magann EF, Terrone DA, Blake PG. 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: 1373-84.
O'Brien JM, Barton JR. Controversies with the diagnosis and management of HELLP syndrome. Clin Obstet Gynecol 2005; 48: 460-77.
Sibai BM. The HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets): much ado about nothing? Am J Obstet Gynecol 1990; 162: 311-6.
van Pampus MG, Wolf H, Westenberg SM, van der Post JA, Bonsel GJ, Treffers PE. 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: 31-6.
Visser W, Wallenburg HCS. Temporising management of severe pre-eclampsia with and without the HELLP syndrome. Br J Obstet Gynaecol. 1995; 102: 111-7.
Geary M. The HELLP syndrome. Br J Obstet Gynaecol. 1997; 104: 887-91.
Barton JR, Sibai BM. Care of the pregnancy complicated by HELLP syndrome. Gastroenterol Clin North Am 1992; 21: 937-50.
Magann EF, Graves GR, Roberts WE, Blake PG, Momson JC, Martin JN Jr. Corticosteroids for enhanced fetal lung maturation in patients with HELLP syndrome: impact on neonates. Aust N Z J Obstet Gynaecol l993; 33: 131-5.
Magann EF, Roberts WE, Perry KG Jr, Chauhan SP, Blake PG, Martin JN Jr. Factors relevant to mode of preterm delivery with syndrome of HELLP (hemolysis, elevated liver enzymes, and low platelets). Am J Obstet Gynecol 1994; 170: 1828-32.
Aarnoudse JG, Houthoff HJ, Weits J, Vellenga E, Huisjes HJ. A syndrome of liver damage and intravascular coagulation in the last trimester of normotensive pregnancy. A clinical and histopathological study. Br J Obstet Gynaecol 1986; 93: 145-55.
Crosby ET. Obstetrical anaesthesia for patients with the syndrome of haemolysis, elevated liver enzymes and low platelets. Can J Anaesth 1991; 38: 227-33.
Martin JN Jr, Blake PG, Perry KG Jr, McCaul JF, Hess LW, Martin RW. The natural history of HELLP syndrome: patterns of disease progression and regression. Am J Obster Gynecol 1991; 164: 1500-9.
Martin JN Jr, Blake PG, Lowry SL, Perry KG Jr, Files JC, Momson JC. 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: 737-41.
Katz VL, Watson WJ, Thorp JM Jr, Hansen W, Bowes WA Jr. Treatment of persistent postpartum HELLP syndrome with plasmapheresis. Am J Perinatol 1992; 9: 120-2.
Magann EF, Bass D, Chauhan SP, Sullivan DL, Martin RW, Martin JN Jr. Antepartum corticosteroids: disease stabilization in patients with the syndrome of hemolysis, elevated liver enzymes, and low platelets (HELLP). Am J Obstet Gynecol 1994; 171: 1148-53.
Magann EF, Perry KG Jr, Meydrech EF, Harris RL, Chauhan SP, Martin JN Jr. Postpartum corticosteroids: accelerated recovery from the syndrome of hemolysis, elevated liver enzymes, and low platelets (HELLP). Am J Obstet Gynecol 1994; 171: 1154-8.
Yalcin OT, Sener T, Hassa H, Ozalp S, Okur A. Effects of postpartum corticosteroids in patients with HELLP syndrome. Int J Gynaecol Obstet 1998; 61: 141-8.
O’Brien JM, Milligan DA, Barton JR. Impact of high-dose corticosteroid therapy for patients with HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome. Am J Obstet Gynecol 2000; 183: 921-4.
Osmanagaoglu MA, Osmanagaoglu S, Ulusoy H, Bozkaya H. Maternal outcome in HELLP syndrome requiring intensive care management in a Turkish hospital. Sao Paulo Med J. 2006; 124: 85-9.

Ayrıntılar

Birincil Dil

İngilizce

Konular

Sağlık Kurumları Yönetimi

Bölüm

Araştırma Makalesi

Yazarlar

Yayımlanma Tarihi

2 Nisan 2009

Gönderilme Tarihi

2 Nisan 2009

Kabul Tarihi

-

Yayımlandığı Sayı

Yıl 2009 Cilt: 31 Sayı: 2

IZ

https://izlik.org/JA79EF36RU

Kaynak Göster

RIS / Bibtex

AMA

1.Yenicesu G, Kol İ, Yenicesu C, Çetin A. HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome. CMJ. 2009;31(2):182-188. https://izlik.org/JA79EF36RU