HELLP syndrome

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HELLP syndrome
Classification and external resources
ICD-10 O14.1
ICD-9 Not assigned
DiseasesDB 30805
MedlinePlus 000890
eMedicine ped/1885
MeSH D017359

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

variant of pre-eclampsia. Both conditions occur during the later stages of pregnancy, or
sometimes after childbirth.

HELLP is an abbreviation of the main findings:[1]

• Hemolytic anemia
• Elevated Liver enzymes and
• Low Platelet count

Contents
[hide]

• 1 Signs and symptoms

• 2 Diagnosis
• 3 Classification
• 4 Pathophysiology
• 5 Treatment
• 6 Epidemiology
• 7 History
• 8 See also

• 9 References

[edit] Signs and symptoms

Often, a patient 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 present after
delivery.

There is gradual but marked onset of headaches (30%), blurred vision, malaise (90%),
nausea/vomiting (30%), "band pain" around the upper abdomen (65%) 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 the patient gets 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,[2] and in 84% when HELLP is complicated by acute renal failure.[3]

Patients who present symptoms of HELLP can be misdiagnosed in the early stages,
increasing the risk of liver failure and morbidity.[4] Rarely, post caesarean patient may
present in shock condition mimicking either pulmonary embolism or reactionary
haemorrhage.

[edit] Diagnosis
In a patient with possible HELLP syndrome, a batch of blood tests is performed: a full
blood count, liver enzymes, renal function and electrolytes and coagulation studies.
Often, fibrin degradation products (FDPs) 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.

A positive D-dimer test in the presence of preeclampsia has recently been reported to be
predictive of patients who will develop HELLP syndrome.[5] D-dimer is a more sensitive
indicator of subclinical coagulopathy and may be positive before coagulation studies are
abnormal.[citation needed]

[edit] Classification
The platelet count has been found to be moderately predictive of severity: under
50,000/mm3 is class I (severe), between 50,000 and 100,000 is class II (moderately
severe) and >100,000 is class III (mild). This system is termed the Mississippi
classification.[6]

[edit] 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 serious challenge.

[edit] 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.

[edit] Epidemiology
Its incidence is reported as 0.2-0.6% of all pregnancies, and 10-20% of women with
comorbid preeclampsia. HELLP usually begins during the third trimester, and usually in
Caucasian women over the age of 25. (Padden, 1999) Rarely cases have been reported as
early as 23 weeks gestation. The outcome for mothers with HELLP syndrome is
generally good. With treatment, maternal mortality is about 1 percent. However
complications have been observed, including placental abruption, acute renal failure,
subcapsular liver hematoma, and retinal detachment.[7]

[edit] History
HELLP syndrome was identified as a distinct clinical entity (as opposed to severe
preeclampsia) by Dr Louis Weinstein in 1982.[1]