HELLP syndrome

From Wikipedia, the free encyclopedia

HELLP syndrome is a
life-threatening obstetric HELLP syndrome
complication usually Classification and external resources
considered to be a variant
Specialty Obstetrics
or complication of
pre-eclampsia.[1] Both ICD-10 O14.2 (http://apps.who.int
conditions usually occur /classifications/icd10/browse
during the later stages of /2015/en#/O14.2)
pregnancy, or sometimes ICD-9-CM 642.5 (http://www.icd9data.com
after childbirth. "HELLP" is /2012/Volume1/630-679
an abbreviation of the
/640-649/642/642.5.htm)
three main features of the
DiseasesDB 30805
syndrome:[2]
(http://www.diseasesdatabase.com
Hemolysis /ddb30805.htm)
Elevated Liver MedlinePlus 000890 (http://www.nlm.nih.gov
enzymes /medlineplus/ency/article
Low Platelet count /000890.htm)
eMedicine ped/1885
(http://www.emedicine.com
Contents /ped/topic1885.htm)
Patient UK HELLP syndrome
1 Signs and (http://patient.info/doctor/hellp-
symptoms syndrome)
2 Pathophysiology MeSH D017359
3 Diagnosis and (https://www.nlm.nih.gov
classification /cgi/mesh
4 Treatment /2015/MB_cgi?field=uid&
5 Prognosis term=D017359)
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 "do not look very sick." [3] Early
symptoms can include:

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

right upper quadrant pain.[3][4]
In 90% of cases, malaise.[4]
In 50% of cases, nausea or vomiting.[4]

There can be gradual but marked onset of headaches (30%), blurred

vision, and paresthesia (tingling in the extremities). 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 in 44% when HELLP is complicated by acute renal
failure.[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. This 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 (DIC), leading to
paradoxical bleeding, which can make emergency surgery a challenge.

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

CoA-dehydrogenase deficiency (LCHAD deficiency) 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. Approximately 80% of infants with LCHAD
deficiency have been born after pregnancies complicated by AFLP or HELLP.
However, what is not known is how many pregnancies complicated by AFLP
or HELLP result in infants with LCHAD deficiency.[8]

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
(FDP) levels are determined, which can be elevated. Lactate dehydrogenase
is a marker of hemolysis and is elevated (> 600 U/liter). Proteinuria is
present but can be mild.

In one 1995 study, a positive D-dimer test in the presence of preeclampsia

was reported to be predictive of woman who will develop HELLP syndrome.
[7][9]

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][10]

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.
Partial HELLP syndrome is characterized by 1-2 features of
HELLP.
Criteria developed at the University of Mississippi, as of 1999:[11]
"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)...."
"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)."[12]

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 DIC 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.[13] 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,[14]

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.[15] In general, however,
factors such as gestational age are more important than the severity of
HELLP in determining the outcome in the baby.[16]

Epidemiology
Its incidence is reported as 0.5-0.9% of all pregnancies, and 10-20% of
women with severe preeclampsia.[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][17]

See also
Acute fatty liver of pregnancy
Hypertrophic decidual vasculopathy

References
1. Haram K, Svendsen E, Abildgaard 3. Weinstein L (Sep 2005). "It has
U (Feb 2009). "The HELLP been a great ride: the history of
syndrome: clinical issues and HELLP syndrome". Am J Obstet
management. A review" Gynecol 193 (3 Pt 1): 860–3.
(http://www.biomedcentral.com doi:10.1016/j.ajog.2005.06.058
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/pdf/1471-2393-9-8.pdf) (PDF). /10.1016%2Fj.ajog.2005.06.058
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(https://dx.doi.org /pubmed/16150288).
/10.1186%2F1471-2393-9-8). 4. Sibai BM (Feb 1990). "The HELLP
PMC 2654858 syndrome (hemolysis, elevated
(https://www.ncbi.nlm.nih.gov liver enzymes, and low platelets):
/pmc/articles/PMC2654858). much ado about nothing?". Am J
PMID 19245695 Obstet Gynecol 162 (2): 311–6.
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5. Sibai BM, Ramadan MK, Usta I, 9. Neiger R, Trofatter MO, Trofatter
Salama M, Mercer BM, Friedman KF Jr (Apr 1995). "D-dimer test
SA (October 1993). "Maternal for early detection of HELLP
morbidity and mortality in 442 syndrome". South Med J 88 (4):
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elevated liver enzymes, and low doi:10.1097/00007611-199504
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%2990043-i). PMID 8238109 AY, Sibai BM (Aug 1996). "Clinical
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1682–7; discussion 1687–90. 6%2970162-x). PMID 8765269
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/10.1016%2F0002-9378%2893 11. Martin JN Jr, Rinehart BK, May
%2990678-c). PMID 8317509 WL, Magann EF, Terrone DA, Blake
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/pubmed/8317509). severe preeclampsia: comparative
7. Padden MO (Sep 1999). "HELLP analysis by HELLP (hemolysis,
syndrome: recognition and elevated liver enzyme levels, and
perinatal management" low platelet count) syndrome
(http://www.aafp.org/afp/1999 classification". Am J Obstet
/0901/p829.html). Am Fam Gynecol 180 (6 Pt 1): 1373–84.
Physician 60 (3): 829–36, 839. doi:10.1016/s0002-9378(99)70
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Categories: Pathology of pregnancy, childbirth and the puerperium

Medical emergencies Syndromes

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