HELLP syndrome is a life-threatening pregnancy complication usually considered to be a

variant or complication of pre-eclampsia.[1] Both conditions usually occur during the later
stages of pregnancy, or sometimes after childbirth. "HELLP" is an abbreviation of the three
main features of the syndrome:[2] Hemolysis, Elevated Liver enzymes, and Low Platelet
count. The syndrome may be associated with serious liver manifestations, including death of
liver cells due to inadequate blood flow and oxygen delivery, bleeding, and rupture.

Contents
 1 Signs and symptoms
 2 Pathophysiology
 3 Diagnosis and classification
 4 Treatment
 5 Prognosis
 6 Epidemiology
 7 History
 8 See also
 9 References

Signs and symptoms

HELLP usually begins during the third trimester; rare cases have been reported as early as 21
weeks gestation. Often, a woman who develops HELLP syndrome has already been followed
up for pregnancy-induced hypertension (gestational hypertension), or is suspected to develop
pre-eclampsia (high blood pressure and proteinuria). Up to 8% of all cases occur after
delivery.

Women with HELLP syndrome often appear non-toxic.[3] Early symptoms can include:

 In 90% of cases, either epigastric pain described as "heartburn" or right upper

quadrant pain develops.[3][4]
 In 90% of cases, malaise occurs.[4]
 In 50% of cases, nausea or vomiting happen.[4]

Gradual but marked onset of headaches (30%), blurred vision, and paresthesia (tingling in the
extremities) can occur. Edema may occur, but its absence does not exclude HELLP
syndrome. Arterial hypertension is a diagnostic requirement, but may be mild. Rupture of the
liver capsule and a resultant hematoma may occur. If a woman has a seizure or coma, the
condition has progressed into full-blown eclampsia.

Disseminated intravascular coagulation is also seen in about 20% of all women with HELLP
syndrome,[5] and in 84% when HELLP is complicated by acute renal failure.[6] Pulmonary
edema is found in 6% of all women with HELLP syndrome,[5] and when HELLP is
complicated by acute renal failure, pulmonary edema is found in 44% of women with the
syndrome.[6]

A woman with symptoms of HELLP can be misdiagnosed in the early stages, increasing the
risk of liver failure and morbidity.[7] Rarely, after a caesarean section surgery, a woman may
have signs and symptoms of a shock condition mimicking either pulmonary embolism or
reactionary haemorrhage.

Pathophysiology
The exact cause of HELLP is unknown, but general activation of the coagulation cascade is
considered the main underlying problem. Fibrin forms crosslinked networks in the small
blood vessels. The formation of these networks leads to a microangiopathic hemolytic
anemia: the mesh causes destruction of red blood cells as if they were being forced through a
strainer. Additionally, platelets are consumed. As the liver appears to be the main site of this
process, downstream liver cells suffer ischemia, leading to periportal necrosis. Other organs
can be similarly affected. HELLP syndrome leads to a variant form of disseminated
intravascular coagulation, leading to paradoxical bleeding, which can make emergency
surgery a challenge.

An association has been demonstrated between long chain long-chain 3-hydroxyacyl-

coenzyme A dehydrogenase deficiency (LCHAD) of the child and maternal HELLP and
acute fatty liver of pregnancy (AFLP). This inherited, autosomal recessive abnormality of
fatty-acid oxidation can result in significant morbidity and mortality in infants, if untreated.
Treatment with dietary manipulation is possible.[8] About 80% of infants with LCHAD
deficiency have been born after pregnancies complicated by AFLP or HELLP. However, how
many pregnancies complicated by AFLP or HELLP result in infants with LCHAD deficiency
is not known.[9]

Diagnosis and classification

HELLP syndrome can be difficult to diagnose due to the variability of symptoms among
pregnant women (frequently a woman will have no symptoms other than general abdominal
pain), and early diagnosis is key in reducing morbidity. If not treated in a timely manner, a
woman can become critically ill or die due to liver rupture/hemorrhage or cerebral edema.

In a woman with possible HELLP syndrome, a batch of blood tests is performed: a full blood
count, a coagulation panel, liver enzymes, electrolytes, and renal function studies. Often,
fibrin degradation product levels are determined, which can be elevated. Lactate
dehydrogenase is a marker of hemolysis and is elevated (>600 U/l). Proteinuria is present but
can be mild.

In one 1995 study, a positive D-dimer test in the presence of pre-eclampsia was reported to
be predictive of woman who will develop HELLP syndrome.[7][10]

The diagnostic criteria for and subtypes of HELLP vary across studies, which "makes
comparison of published data difficult."[1] The classifications include:

 Criteria developed at the University of Tennessee:[4][11]

o HELLP is characterized by hemolysis on peripheral blood smear with serum
lactate dehydrogenase >600 IU/l; serum aspartate aminotransferase >70 IU/l;
and platelet count <100,000/μl.
o Partial HELLP syndrome is characterized by one or two features of HELLP.
 Criteria developed at the University of Mississippi, as of 1999:[12]
 o "The diagnosis of HELLP syndrome required the presence of
thrombocytopenia (perinatal platelet nadir ≤150,000 cells/μl), evidence of
hepatic dysfunction (increased aspartate aminotransferase level of ≥40 IU/l,
increased alanine aminotransferase level of ≥40 IU/l, or both, with increased
lactate dehydrogenase (LDH) level of ≥600 IU/l), and evidence of hemolysis
(increased LDH level, progressive anemia)...."
o "Class 1 HELLP syndrome featured severe thrombocytopenia with a platelet
nadir of ≤50,000 cells/μl, class 2 HELLP syndrome featured moderate
thrombocytopenia with a platelet nadir between >50,000 and ≤100,000
cells/μl, and class 3 HELLP syndrome featured mild thrombocytopenia with a
platelet nadir between >100,000 and ≤150,000 cells/μl."
 Criteria developed at the University of Mississippi, as of 2006: "For a patient to merit
a diagnosis of HELLP syndrome, class 1 requires severe thrombocytopenia (platelets
≤50,000/μl), evidence of hepatic dysfunction (AST [aspartate aminotransferase]
and/or ALT [alanine aminotransferase] ≥70 IU/l), and evidence suggestive of
hemolysis (total serum LDH ≥600 IU/l); class 2 requires similar criteria except
thrombocytopenia is moderate (>50,000 to ≤100,000/μl); and class 3 includes patients
with mild thrombocytopenia (platelets >100,000 but ≤150,000/μl), mild hepatic
dysfunction (AST and/or ALT ≥40 IU/l), and hemolysis (total serum LDH ≥600
IU/L)."[13]

Treatment
The only effective treatment is prompt delivery of the baby. Several medications have been
investigated for the treatment of HELLP syndrome, but evidence is conflicting as to whether
magnesium sulfate decreases the risk of seizures and progress to eclampsia. The disseminated
intravascular coagulation is treated with fresh frozen plasma to replenish the coagulation
proteins, and the anemia may require blood transfusion. In mild cases, corticosteroids and
antihypertensives (labetalol, hydralazine, nifedipine) may be sufficient. Intravenous fluids are
generally required. Hepatic hemorrhage can be treated with embolization, as well, if life-
threatening bleeding ensues.

The University of Mississippi standard protocol for HELLP includes corticosteroids.[14]

However, a 2009 review found "no conclusive evidence" supporting corticosteroid therapy,[1]
and a 2010 systematic review by the Cochrane Collaboration also found "no clear evidence of
any effect of corticosteroids on substantive clinical outcomes" either for the mothers or for
the newborns,[15]

Prognosis
With treatment, maternal mortality is about 1 percent, although complications such as
placental abruption, acute renal failure, subcapsular liver hematoma, permanent liver damage,
and retinal detachment occur in about 25% of women.[5] Perinatal mortality (stillbirths plus
death in infancy) is between 73 and 119 per 1000 babies of woman with HELLP, while up to
40% are small for gestational age.[16] In general, however, factors such as gestational age are
more important than the severity of HELLP in determining the outcome in the baby.[17]

Epidemiology
Its incidence is reported as 0.5-0.9% of all pregnancies, and 10-20% of women with severe
pre-eclampsia.[1] HELLP usually occurs in Caucasian women over the age of 25.[7]

History
HELLP syndrome was identified as a distinct clinical entity (as opposed to severe pre-
eclampsia) by Dr. Louis Weinstein in 1982.[2] In a 2005 article, Weinstein wrote that the
unexplained postpartum death of a woman who had hemolysis, abnormal liver function,
thrombocytopenia, and hypoglycemia motivated him to review the medical literature and to
compile information on similar women.[3] He noted that cases with features of HELLP had
been reported as early as 1954.[3][18]
Sindrom HELLP adalah komplikasi kehamilan yang mengancam jiwa yang biasanya dianggap sebagai
varian atau komplikasi preeklampsia. [1] Kedua kondisi biasanya terjadi pada tahap akhir kehamilan,
atau kadang setelah melahirkan. "HELLP" adalah singkatan dari tiga ciri utama sindrom ini: [2]
Hemolisis, enzim Hati yang meningkat, dan jumlah Platelet Rendah. Sindrom ini mungkin terkait
dengan manifestasi hati yang serius, termasuk kematian sel hati karena aliran darah dan persalinan,
perdarahan, dan ruptur yang tidak adekuat.

Isi

1 Tanda dan gejala

2 Patofisiologi
3 Diagnosis dan klasifikasi
4 Pengobatan
5 Prognosis
6 Epidemiologi
7 Sejarah
8 Lihat juga
9 Referensi

Tanda dan gejala

HELLP biasanya dimulai pada trimester ketiga; Kasus langka telah dilaporkan pada usia kehamilan 21
minggu. Seringkali, seorang wanita yang mengembangkan sindrom HELLP telah ditindaklanjuti untuk
hipertensi yang diinduksi kehamilan (gestational hypertension), atau diduga mengembangkan
preeklampsia (tekanan darah tinggi dan proteinuria). Sampai 8% dari semua kasus terjadi setelah
melahirkan.

Wanita dengan sindrom HELLP sering tampak tidak beracun. [3] Gejala awal bisa meliputi:

Pada 90% kasus, nyeri epigastrik yang digambarkan sebagai "heartburn" atau nyeri kuadran kanan
atas berkembang. [3] [4]
Pada 90% kasus, malaise terjadi. [4]
Pada 50% kasus, mual atau muntah terjadi. [4]

Sakit kepala yang bertahap namun ditandai (30%), penglihatan kabur, dan paresthesia (kesemutan di
ekstremitas) dapat terjadi. Edema bisa terjadi, namun ketiadaannya tidak mengecualikan sindrom
HELLP. Hipertensi arterial adalah persyaratan diagnostik, namun mungkin ringan. Pecahnya kapsul
hati dan hematoma resultan bisa terjadi. Jika seorang wanita mengalami kejang atau koma,
kondisinya telah berkembang menjadi eklampsia penuh.

Koagulasi intravaskular diseminata juga terlihat pada sekitar 20% wanita dengan sindrom HELLP, [5]
dan 84% ketika HELLP dipersulit oleh gagal ginjal akut. [6] Edema paru ditemukan pada 6% wanita
dengan sindrom HELLP, [5] dan ketika HELLP dipersulit oleh gagal ginjal akut, edema paru ditemukan
pada 44% wanita dengan sindrom ini. [6]
Seorang wanita dengan gejala HELLP dapat salah didiagnosis pada tahap awal, meningkatkan risiko
gagal hati dan morbiditas. [7] Jarang, setelah operasi caesar, seorang wanita mungkin memiliki tanda
dan gejala kondisi kejut yang meniru embolisme pulmonal atau perdarahan reaksioner.
Patofisiologi

Penyebab pasti HELLP tidak diketahui, namun aktivasi umum dari kaskade koagulasi dianggap
sebagai masalah mendasar. Fibrin membentuk jaringan silang dalam pembuluh darah kecil.
Pembentukan jaringan ini menyebabkan anemia hemolitik mikroangiopati: jala menyebabkan
kerusakan sel darah merah seolah-olah dipaksakan melalui saringan. Selain itu, platelet dikonsumsi.
Sebagai hati tampaknya menjadi situs utama dari proses ini, sel-sel hati hilir menderita iskemia, yang
menyebabkan nekrosis periportal. Organ-organ lain bisa juga terpengaruh. Sindrom HELLP mengarah
pada bentuk varian dari koagulasi intravaskular diseminata, yang menyebabkan perdarahan
paradoks, yang dapat membuat operasi darurat menjadi tantangan.

Sebuah asosiasi telah ditunjukkan antara defisiensi defisiensi 3-hidroksyacyl-coenzyme A

dehidrogenase rantai panjang rantai panjang dan anak ibu dan hati berlemak akut kehamilan (AFLP).
Ini mewarisi, kelainan resesif autosomal oksidasi asam lemak dapat menyebabkan morbiditas dan
mortalitas yang signifikan pada bayi, jika tidak diobati. Pengobatan dengan manipulasi diet adalah
mungkin. [8] Sekitar 80% bayi dengan defisiensi LCHAD telah lahir setelah kehamilan yang dipersulit
oleh AFLP atau HELLP. Namun, berapa banyak kehamilan yang dipersulit oleh AFLP atau HELLP
menyebabkan bayi kekurangan LCHAD tidak diketahui. [9]
Diagnosis dan klasifikasi

Sindrom HELLP sulit untuk didiagnosis karena variabilitas gejala di antara wanita hamil (seringkali
seorang wanita tidak akan memiliki gejala selain sakit perut secara umum), dan diagnosis dini adalah
kunci untuk mengurangi morbiditas. Jika tidak diobati secara tepat waktu, seorang wanita bisa
menjadi sakit kritis atau meninggal karena pecahnya hati / pendarahan atau edema serebral.

Pada wanita dengan sindrom HELLP yang mungkin, serangkaian tes darah dilakukan: hitung darah
penuh, panel koagulasi, enzim hati, elektrolit, dan fungsi fungsi ginjal. Seringkali, tingkat produk
degradasi fibrin ditentukan, yang dapat ditingkatkan. Laktat dehidrogenase adalah penanda
hemolisis dan meningkat (> 600 U / l). Proteinuria hadir tapi bisa ringan.

Dalam satu penelitian 1995, tes D-dimer positif di hadapan pre-eklampsia dilaporkan menjadi
prediktif terhadap wanita yang akan mengembangkan sindrom HELLP. [7] [10]

Kriteria diagnostik untuk dan subtipe HELLP bervariasi di seluruh penelitian, yang "membuat
perbandingan data yang dipublikasikan sulit." [1] Klasifikasinya meliputi:

Kriteria dikembangkan di University of Tennessee: [4] [11]

HELLP dicirikan oleh dia