HELLP Syndrome: Recognition and Perinatal Management

HELLP Syndrome: Recognition and Perinatal Management

HELLP Syndrome Dr. Khosrou Naghibi HELLP 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. 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. Epidemiology and Risk Factors HELLP syndrome 0.2 to 0.6 % of all pregnancies. Preeclampsia 5 to 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 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. 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. 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.

Clinical Presentation 90%of patients present with generalized malaise, 65 % with epigastric pain, 30 % with nausea and vomiting, 31 percent with headache. All are nonspecific symptoms 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 In one retrospective chart review of patients with HELLP syndrome, only two of 14 patients entered the hospital with the

correct diagnosis. 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.

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. Clinical Presentation 90

90 80 80 70 70 60 60 50 50 40 40

30 30 20 20 10 10 00 90 90

SYMPTOMS 65 65 general generalmalase malase epigastric epigastricpain pain 30 30

symptoms symptoms 31 31 vomiting vomiting haedache haedache

Clinical Presentation 90 90 signs 80 70 60

Rt.hypochond.pain 50 40 30 30 edema

hypertention + proteinuria 20 10 0 30 signs 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.

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 > 70U/L Lactate dehydrogenase level >600 U/L

Low platelet count Platelet count < 150 000/mm3 Platelet count appears to be the most reliable indicator of the presence of HELLP syndrome

Classification on the basis of platelet count class I, less than 50,000 per mm3 class II, 50,000 to less than 100,000 per mm3 class III, 100,000 to 150,000 per mm3 Management Delivery Corticosteroids Magnesium sulphate

Hypotensive drugs Blood products 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 Eligibility to conservative management hypertension is controlled at less than 160/110 mm hg,

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 dexamethasone (Decadron) in a high dosage of 10 mg intravenously every 12 hours has been The antenatal administration of

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 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. Intravenously administered dexamethasone appears to be more effective than intramuscularly adminstered betamethasone for the antepartum treatment of mothers with HELLP syndrome. (Am J Obstet Gynecol 2001;184:1332-9.).

Patients with HELLP syndrome should be treated prophylactically with magnesium sulfate to prevent seizures, whether hypertension is present or not. 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. The most commonly used antihypertensive agent has been hydralazine Labetolol Nifedipine

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. 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. 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 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 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 Complications 60% 60.00% 50.00% 40.00% 25%

30.00% 20.00% 10.00% 0.00% 1.10% matern.mort. maternal complication

fetal complication Hellp syn The incidence of hemorrhagic complications is higher when platelet counts are < 40,000 per mm3. 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.

by three to four days postpartum The laboratory abnormalities in HELLP syndrome typically worsen after delivery and then begin to resolve.

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