Professional Documents
Culture Documents
Sindrome Hellp
Sindrome Hellp
HYPERTENSION, ELEVATED
LIVER ENZYMES, AND LOW
PLATELETS (HELLP)
ALI CETIN, MD
Pathophysiology
HELLP syndrome is a specific manifestation of endothelial dysfunction during pregnancy. It is unknown why
some preeclamptic women develop HELLP syndrome
and others do not.5 The pathophysiologic process that
underlies HELLP syndrome appears to be microvascular
damage with endothelial injury and organ hypoperfusion. Microangiopathic hemolytic anemia is thought to
result from the passage of red blood cells through small
blood vessels with damaged intima and fibrin deposition,
leading to the appearance on peripheral smear of triangular cells, burr cells, echinocytes, and spherocytes.6
The extensive platelet activation can result in thrombocytopenia, defined as a platelet count < 100,000/;gmL.
Severe thrombocytopenia (< 50,000/;gmL) may impair
coagulation. Severe derangement of coagulation induced
by thrombotic microangiopathic anemia may in some
cases result in disseminated intravascular coagulation
(DIC). However, DIC is a distinct, different disorder.5
Periportal and/or focal parenchymal lesions with large
fibrin deposits are histopathologic abnormalities of
HELLP syndrome.7 As a result, serum liver enzymes such
as aspartate aminotransaminase (AST) and alanine
aminotransaminase (ALT) are elevated, although changes
in AST and ALT concentrations in plasma can also reflect
extrahepatic (particularly erythrocyte) rather than
hepatic damage.8 Class alpha glutathione S-transferases
HELLP / 417
Presentation
Patients with HELLP syndrome exhibit the various signs
and symptoms commonly observed with preeclampsia.
These signs and symptoms may also be seen in women
with severe preeclampsia and eclampsia who do not have
HELLP syndrome. 6 Although HELLP syndrome
commonly has a slow initial phase followed by an accelerative phase that can result in multiorgan failure without intervention, sometimes disease expression can be
explosive. Multiple organs are affected, most often the
liver, kidneys, central nervous system, and the hematologic system.6,11 Women with HELLP syndrome usually
present remote from term with complaints of epigastric
or right upper quadrant pain. 6,12 Some women with
HELLP syndrome experience nausea or vomiting,
whereas other women with the syndrome experience
nonspecific viral-syndrome-like symptoms. 13 Most
women with HELLP syndrome present with a history of
malaise a few days prior to presentation. The most
common symptoms reported by Weinstein1 were nausea
and/or vomiting and epigastric pain. Right upper quadrant or epigastric pain is thought to be caused by
obstruction of blood flow in the hepatic sinusoids, which
are blocked by intravascular fibrin deposits.
HELLP syndrome has a slow initial phase followed
by an accelerative phase that can result in multiorgan failure without intervention.
The physical examination may be normal in patients
with HELLP syndrome. However, right upper quadrant
tenderness is present in as many as 90% of affected
women.13 Edema is not a useful marker because swelling
is a factor in up to 30% of normal pregnancies.
Hypertension and proteinuria may be absent or mild.
The differential diagnosis of HELLP syndrome includes
acute fatty liver of pregnancy, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome.
Acute renal failure, cerebral edema, and hemorrhage,
eclampsia, and placental abruption are among the other
most feared complications associated with HELLP
syndrome.5
Evaluation
The diagnostic criteria used for HELLP syndrome are
variable and inconsistent.14 The diagnosis of HELLP
syndrome is based on the laborator y evidence of
microangiopathic hemolytic anemia, EL, and thrombocytopenia in the pregnant or puerperal patient with other
signs and symptoms that are consistent with a diagnosis
of preeclampsia or eclampsia.
Diagnosis of HELLP syndrome is based on the laboratory evidence of microangiopathic hemolytic
anemia, EL, and thrombocytopenia.
HEMOLYSIS
Hemolysis, defined as the presence of microangiopathic
hemolytic anemia, is the hallmark of the triad of HELLP
syndrome.13 It is confirmed by the sensitive, but not
specific, observation of burr cells (crenated, contracted,
distorted red blood cells with spiny projections along the
periphery); schistocytes (small, irregularly shaped red
blood cell fragments); polychromasia on peripheral
blood smear examination; elevated LDH levels; and
significant drop in hemoglobin levels. Red blood cell
fragmentation occurs secondary to passage through small
blood vessels with intimal damage and fibrin deposition.
Hemolysis is more common than realized, as automated
technology has resulted in a decrease of manual examination of peripheral smears. LDH levels, measurements
of indirect bilirubin, and low serum haptoglobin levels
have also been used as additional markers of evidence for
hemolysis. A significant percentage of published reports
included patients without the findings of hemolysis;
hence, these patients may fit the criteria for HELLP
syndrome.6,15
THROMBOCYTOPENIA
LP is the third abnormality required to establish the diagnosis of HELLP syndrome. There is no consensus among
Management
The goals of management of women with HELLP
syndrome include prevention of maternal morbidity and
mortality, and reduction of perinatal morbidity and
mortality. The clinical course of women with HELLP
syndrome is characterized by usually progressive and
sometimes sudden deterioration in maternal and fetal
conditions.16 Therefore, patients with suspected diagnosis of HELLP syndrome should be hospitalized immediately and observed in a labor and delivery unit. Patients
with HELLP syndrome who are remote from term
should be referred to a tertiary care center.6
Optimal maternal and fetal outcome are dependent
on prompt recognition and treatment of HELLP
syndrome.
There is no consensus on the management of HELLP
syndrome.12 Some authors consider its presence to be an
indication for immediate delivery by cesarean section,
whereas others recommend a more conservative
approach to prolong pregnancy in cases of fetal immaturity. Consequently, the literature describes several thera-
CORTICOSTEROID THERAPY
Administration of corticosteroids to women with HELLP
syndrome in the preterm period improves maternal
outcome by accelerating the recovery of biochemical
abnormalities and clinical signs. The stabilizing effect of
corticosteroids on activated endothelial cells and
platelets, as well as the antiinflammator y and
immunomodulative properties, may account for the
HELLP / 419
Outcome
Despite their similarities, HELLP syndrome is associated
with significantly greater maternal and fetal morbidity
and mortality than preeclampsia.21,22 Maternal mortality
rates vary from 0 to 24%.23 In a review of 54 maternal
deaths associated with HELLP syndrome, a delay in diagnosis was noted in 51%, with one-third the result of
patient delay, and the other two-thirds resulting from
delay in physician diagnosis. 24 Patients with HELLP
syndrome are at increased risk for DIC, abruptio placentae, acute renal failure, pulmonary edema and pleural
effusions, acute respiratory distress syndrome, liver
infarcts, and ruptured liver hematomas. Perinatal mortality associated with HELLP syndrome has been estimated
as 11%.25 Most of the perinatal complications seemed to
be the result of prematurity and placental insufficiency.
Overall, the risk for preeclampsia in a subsequent
pregnancy was 21%, but the recurrence rate of HELLP
syndrome was only 4%.24
Prevention
Until the pathogenesis of preeclampsia is well defined, it
is doubtful that we will find therapies to prevent
preeclampsia and HELLP syndrome. With improved
prenatal care, early detection of signs and symptoms of
preeclampsia and continuous education of both patients
and healthcare providers regarding the prompt reporting
and response to serious symptoms such as severe
headaches, blurred vision, mental status changes, and
epigastric pain with nausea and vomiting during pregnancy and puerperal period.
References
1. Weinstein L. Syndrome of hemolysis, elevated liver enzymes,
and low platelet count: a severe consequence of hypertension in pregnancy. Am J Obstet Gynecol 1982;142:15967.