HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
HELLP Syndrome - Recognition and Management Tutorial [1] | August 17, 2011 By Mohammed Abdalla, MD [2] HELLP Syndrome as a Separate Entity Dr. Mohammed Abdalla EGYPT, Domiat G. HospitalHELLP Syndrome May it be a separate entity? yes HELLP, a syndrome characterized by hemolysis, elevated liver enzyme levels and a low platelet count, is an obstetric complication that is frequently misdiagnosed at initial presentation. Many investigators consider the syndrome to be a variant of preeclampsia, but it may be a separate entity.
HELLP Syndrome as a Separate Entity Dr. Mohammed Abdalla EGYPT, Domiat G. Hospital
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
HELLP Syndrome May it be a separate entity? yes
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
HELLP, a syndrome characterized by hemolysis, elevated liver enzyme levels and a low platelet count, is an obstetric complication that is frequently misdiagnosed at initial presentation. Many investigators consider the syndrome to be a variant of preeclampsia, but it may be a separate entity.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
In some cases, HELLP symptoms are the first warning of preeclampsia and the condition is misdiagnosed as hepatitis, idiopathic thrombocytopenic purpura, gallbladder disease, or thrombotic thrombocytopenic purpura.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Epidemiology and Risk Factors • HELLP syndrome 0.2 to 0.6% of all pregnancies. • Preeclampsia 5 - 7% of all pregnancies. • Superimposed HELLP syndrome develops in 4 to 12 percent of women with preeclampsia or eclampsia. • Maternal mortality has been estimated to be as high as 2 - 24% • Perinatal mortality is equally high, ranging from 9 - 39% Wolf JL. Liver disease in pregnancy. Med Clin North Am 1996.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Etiology and Pathogenesis • The hemolysis in HELLP syndrome is a microangiopathic hemolytic anemia. Red blood cells become fragmented as they pass through small blood vessels with endothelial damage and fibrin deposits. • The peripheral smear may reveal spherocytes, schistocytes, triangular cells and burr cells. • Increase in Bilirubin and lactic dehydrogenase levels.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Etiology and Pathogenesis The elevated liver enzyme levels in the syndrome are thought to be secondary to obstruction of hepatic blood flow by fibrin deposits in the sinusoids. This obstruction leads to periportal necrosis and, in severe cases, intrahepatic hemorrhage, subcapsular hematoma formation or hepatic rupture.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Etiology and Pathogenesis The thrombocytopenia has been attributed to increased consumption and/or destruction of platelets. With platelet activation, thromboxane A and serotonin are released, causing vasospasm, platelet agglutination and aggregation, and further endothelial damage.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Clinical Presentation • 90% of patients present with generalized malaise, • 65% with epigastric pain, • 30% with nausea and vomiting, • 31% with headache. All are nonspecific symptoms
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Because of the variable nature of the clinical presentation, the diagnosis of HELLP syndrome is generally delayed for an average of eight days. Usually presented by complications
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
In one retrospective chart review of patients with HELLP syndrome, only two of 14 patients entered the hospital with the correct diagnosis.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Because early diagnosis of this syndrome is critical, any pregnant woman who presents with malaise or a viral-type illness in the third trimester should be evaluated with a complete blood cell count and liver function tests.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Clinical Presentation The physical examination may be normal in patients with HELLP syndrome. 1) Right upper quadrant tenderness 90% 2) Edema is not a useful marker 3) Hypertension and proteinuria may be absent or mild.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Clinical Presentation
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Clinical Presentation
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Diagnosis There is agreement among most of the authors that, the diagnosis requires the concurrence of hemolysis, elevated liver enzymes, and low platelet count. However, there is obviously still a lack of consensus on the laboratory parameters and their cutoff values used to diagnose. Martin JN Jr, Rinehart BK, May WL, Magann EF, Terrone DA, Blake PG
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Laboratory Diagnostic Criteria for HELLP syndrome Haemolysis Abnormal peripheral smear: spherocytes, schistocytes, triangular cells and burr cells Total Bilirubin level > 1.2 mg/dL Lactate dehydrogenase level > 600U/L Elevated liver function test result Serum aspartate amino transferase level > 70 U/L Lactate dehydrogenase level > 600 U/L Low platelet count Platelet count < 150 000/mm3
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Platelet count appears to be the most reliable indicator of the presence of HELLP syndrome
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Clinical utility of strict diagnostic criteria for the HELLP The use of strict diagnostic criteria in the definition of the HELLP syndrome allows for greater prediction of complication rates. and define the cases that are eligible to conservative management.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Classification Based on the number of abnormalities.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Classification on the basis of platelet count class I, less than 50,000 per 3mm class II, 50,000 to less than 100,000 per mm3 class III, 100,000 to 150,000 per mm3
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Management Delivery Corticosteroids Magnesium sulphate Hypotensive drugs Blood products
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
The treatment approach should be based on the estimated gestational age and the condition of the mother and fetus. Prolongation of pregnancy, in theory, may be favourable for the foetus whereas it remains controversial whether maternal condition is further deteriorated by expectant management. Visser W, Wallenburg HC. Temporising management of severe pre-eclampsia with and without the HELLP syndrome. Br J Obstet Gynaecol 1995;102:111-7
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Eligibility to conservative management • Hypertension is controlled at less than • Oliguria responds to fluid management. • Elevated liver function values are not associated with right upper quadrant or epigastric pain. • Class II – III (platelet count) > 50000
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Corticosteroids
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
The Cochrane Library holds two protocols which when complete may summarize evidence to date on the use of corticosteroids for HELLP syndrome and interventionist versus expectant management of severe pre-eclampsia before term.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
The antenatal administration of dexamethasone (Decadron) in a high dosage of 10 mg intravenously every 12 hours has been shown to markedly improve the laboratory abnormalities associated with HELLP syndrome. Steroids given antenatally do not prevent the typical worsening of laboratory abnormalities after delivery. However, laboratory abnormalities resolve more quickly in patients who continue to receive steroids postpartum. Magann EF, Bass D, Chauhan SP, Sullivan DL, Martin RW, Martin JN Jr. Am J Obstet Gynecol 1994;171:1148-53.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Corticosteroid therapy should be instituted in patients with HELLP syndrome who have a platelet count of less than 100,000 per mm3 and should be continued until liver function abnormalities are resolving and the platelet count is greater than 100,000 per mm3. Magann EF, Perry KG Jr, Meydrech EF, Harris RL, Chauhan SP, Martin JN Jr. Am J Obstet Gynecol 1994;171:1154-8.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Intravenously administered dexamethasone appears to be more effective than intramuscularly administered betamethasone for the antepartum treatment of mothers with HELLP syndrome. (Am J Obstet Gynecol 2001;184:1332-9.)
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Administration of glucocorticoids increases the use of regional anesthesia in women with antepartum HELLP syndrome who have thrombocytopenia. (Am J Obstet Gynecol 2002;186:475-9.)
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Patients treated with dexamethasone exhibit longer time to delivery. This facilitates maternal transfer to a tertiary care center and postnatal maturity of fetal lungs. (Am J Obstet Gynecol 2002;186:475-9.)
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Magnesium Sulphate
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Patients with HELLP syndrome should be treated prophylactically with magnesium sulfate to prevent seizures, whether hypertension is present or not.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Antihypertensive Agent
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Antihypertensive therapy should be initiated if blood pressure is consistently greater than 160/110 mm hg despite the use of magnesium sulfate. The goal is to maintain diastolic blood pressure between 90 and 100 mm hg.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
The most commonly used antihypertensive agent has been: Hydralazine Labetolol Nifedipine
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Blood Products
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Between 38 -93 % of patients with HELLP syndrome receive some form of blood product. Patients with a platelet count greater than 40,000 per mm3 are unlikely to bleed.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
• • Patients who undergo cesarean section should be transfused if their platelet count is less than 50,000 per mm3. • Prophylactic transfusion of platelets at delivery does not reduce the incidence of postpartum hemorrhage or hasten normalization of the platelet count. • Patients with DIC should be given fresh frozen plasma and packed red blood cells.
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Anesthesia Considerations
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Pain relief with intravenous narcotics and local anesthesia is acceptable but certainly not optimal for pain control. Epidural anesthesia has been controversial but it is the technique of choice when it can be accomplished safely. Insertion of an epidural catheter is generally safe in patients with a platelet count greater than 100,000 per mm3. General anesthesia can be used when regional anesthesia is considered unsafe. Portis R, Jacobs MA, Skerman JH, Skerman EB. HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) pathophysiology and anesthetic considerations. AANA J 1997; 65:37-47.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Complications
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Complications • The mortality rate for women with HELLP syndrome is approximately 1.1 % • From 1 to 25 % of affected women develop serious complications such as DIC, placental abruption, adult respiratory distress syndrome, hepatorenal failure, pulmonary edema, subcapsular hematoma and hepatic rupture. • A significant percentage of patients receive blood products. Sibai BM, Ramadan MK, Usta I, Salama M, Mercer BM, Friedman SA. Maternal morbidity and mortality in 442 pregnancies with hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome). Am J Obstet Gynecol 1993;169:1000-6
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Complications • Infant morbidity and mortality rates range from 10 to 60 %, depending on the severity of maternal disease. • Infants affected by HELLP syndrome are more likely to experience intrauterine growth retardation and respiratory distress syndrome. Dotsch J, Hohmann M, Kuhl PG. Neonatal morbidity and mortality associated with maternal haemolysis, elevated liver enzymes and low platelets syndrome. Eur J Pediatr 1997;156:389-91.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Complications
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Take Home Once the diagnosis of HELLP syndrome has been established, the best markers to follow are the maternal lactate dehydrogenase level and the maternal platelet count
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Take Home The laboratory abnormalities in HELLP syndrome typically worsen after delivery and then begin to resolve by three to four days postpartum. Martin JN Jr, Blake PG, Perry KG Jr, McCaul JF, Hess LW, Martin RW. The natural history of HELLP syndrome: patterns of disease progression and regression. Am J Obstet Gynecol 1991;164(6 pt 1):1500-9.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Take Home The incidence of hemorrhagic complications is higher when platelet counts are < 40,000 per mm3.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Take Home Patients with HELLP syndrome who complain of severe right upper quadrant pain, neck pain or shoulder pain should be considered for hepatic imaging regardless of the severity of the laboratory abnormalities, to assess for subcapsular haematoma or rupture.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Take Home Patients with HELLP syndrome should be routinely treated with corticosteroids.
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HELLP Syndrome - Recognition and Management Published on Diagnostic Imaging (http://www.diagnosticimaging.com)
Thank you
Source URL: http://www.diagnosticimaging.com/tutorial/hellp-syndrome-recognition-and-management Links: [1] http://www.diagnosticimaging.com/tutorial [2] http://www.diagnosticimaging.com/authors/mohammed-abdalla-md
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