HELLP syndrome

DiseaseDisorder infobox
Name = HELLP syndrome
ICD10 = ICD10|O|14|1|o|10
ICD9 = "Not assigned"
ICDO =


Caption =
OMIM =
OMIM_mult =
MedlinePlus = 000890
eMedicineSubj = ped
eMedicineTopic = 1885
DiseasesDB = 30805
MeshID = 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:cite journal |author=Weinstein L |title=Syndrome of hemolysis, elevated liver enzymes, and low platelet count: a severe consequence of hypertension in pregnancy |journal=Am. J. Obstet. Gynecol. |volume=142 |issue=2 |pages=159–67 |year=1982 |pmid=7055180 |doi=]
* Hemolytic anemia
* Elevated Liver enzymes and
* Low Platelet count

igns 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, [Sibai, BM. Maternal morbidity and mortality in 442 pregnancies with HELLP syndrome. Am J Obstet Gynecol 1993; 169:1000.] and in 84% when HELLP is complicated by acute renal failure. [Sibai, BM. Acute renal failure in pregnancies complicated by HELLP. Am J Obstet Gynecol 1993; 168:1682.]

Patients who present symptoms of HELLP can be misdiagnosed in the early stages, increasing the risk of liver failure and morbidity. [cite journal |author=Padden MO |title=HELLP syndrome: recognition and perinatal management |journal=American family physician |volume=60 |issue=3 |pages=829–36, 839 |year=1999 |pmid=10498110 |doi= ] Rarely, post caesarean patient may present in shock condition mimicking either pulmonary embolism or reactionary hemorrhage.

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.cite journal |author=Padden MO |title=HELLP syndrome: recognition and perinatal management |journal=American family physician |volume=60 |issue=3 |pages=829–36, 839 |year=1999 |pmid=10498110 |doi=] D-dimer is a more sensitive indicator of subclinical coagulopathy and may be positive before coagulation studies are abnormal.Fact|date=August 2007

Classification

The platelet count has been found to be moderately predictive of severity: under 50 million/L is class I (severe), between 50 and 100 is class II (moderately severe) and >100 is class III (mild). This system is termed the Mississippi classification. [cite journal |author=Martin JN, Blake PG, Lowry SL, Perry KG, Files JC, Morrison JC |title=Pregnancy complicated by preeclampsia-eclampsia with the syndrome of hemolysis, elevated liver enzymes, and low platelet count: how rapid is postpartum recovery? |journal=Obstetrics and gynecology |volume=76 |issue=5 Pt 1 |pages=737–41 |year=1990 |pmid=2216215 |doi=]

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.

Treatment

The only effective treatment is 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.

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 abruptio placentae, acute renal failure, subcapsular liver hematoma, and retinal detachment. [cite journal |author=Sibai BM, Ramadan MK, Usta I, Salama M, Mercer BM, Friedman SA |title=Maternal morbidity and mortality in 442 pregnancies with hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome) |journal=Am. J. Obstet. Gynecol. |volume=169 |issue=4 |pages=1000–6 |year=1993 |pmid=8238109]

History

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

ee also

*Acute fatty liver of pregnancy

References