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Diagnosis and treatment of fulminant HELLP syndrome
SHEN Jian, WANG Zhi-jian
Chinese Journal of Practical Gynecology and Obstetrics ›› 2025, Vol. 41 ›› Issue (9) : 869-872.
PDF(882 KB)
PDF(882 KB)
Diagnosis and treatment of fulminant HELLP syndrome
HELLP syndrome(hemolysis,elevated liver enzymes,and thrombocytopenia syndrome)is a severe pregnancy-related complication characterized by high maternal and fetal mortality rates.Fulminant HELLP syndrome,a critical subtype of HELLP syndrome,is marked by rapid disease progression,significantly abnormal laboratory indices(e.g.,AST>2000 U/L or LDH>3000 U/L),and multi-organ dysfunction.This article reviews the pathophysiological mechanisms,clinical manifestations,diagnostic criteria,and therapeutic principles of fulminant HELLP syndrome.
HELLP syndrome / fulminant / pregnancy complications / multidisciplinary management
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No authors listed. Gestational Hypertension and Preeclampsia:ACOG Practice Bulletin, Number 222[J]. Obstet Gynecol, 2020, 135(6):e237-e260.DOI:10.1097/AOG.0000000000003891.
Hypertensive disorders of pregnancy constitute one of the leading causes of maternal and perinatal mortality worldwide. It has been estimated that preeclampsia complicates 2–8% of pregnancies globally (1). In Latin America and the Caribbean, hypertensive disorders are responsible for almost 26% of maternal deaths, whereas in Africa and Asia they contribute to 9% of deaths. Although maternal mortality is much lower in high-income countries than in developing countries, 16% of maternal deaths can be attributed to hypertensive disorders (1, 2). In the United States, the rate of preeclampsia increased by 25% between 1987 and 2004 (3). Moreover, in comparison with women giving birth in 1980, those giving birth in 2003 were at 6.7-fold increased risk of severe preeclampsia (4). This complication is costly: one study reported that in 2012 in the United States, the estimated cost of preeclampsia within the first 12 months of delivery was $2.18 billion ($1.03 billion for women and $1.15 billion for infants), which was disproportionately borne by premature births (5). This Practice Bulletin will provide guidelines for the diagnosis and management of gestational hypertension and preeclampsia.
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We assessed the incidence, risk factors and adverse birth outcomes associated with elevated liver enzymes and low platelets (HELLP) syndrome.
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To describe the presentation, outcomes, and management strategies for cases of subcapsular liver hematoma associated with preeclampsia, eclampsia, or HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome.This was a case series of individuals with subcapsular liver hematoma managed at a single level IV center over a 10-year period, from 2013 to 2024. Presenting signs and symptoms, laboratory findings, time of onset, management strategies, acute perinatal and maternal outcomes, and long-term outcomes such as subsequent pregnancies were reviewed in the medical record and recorded. Data were summarized using descriptive statistics, with results reported as means, standard deviations, and ranges. Categorical variables were summarized as counts and percentages.There were 13 cases of subcapsular liver hematoma associated with preeclampsia, eclampsia, and HELLP syndrome between 2013 and 2024. In 10 of the 13 pregnancies (76.9%), delivery was preterm. The most common presenting symptoms were epigastric or right upper quadrant pain (53.8%), followed by abdominal distention (38.5%). Diagnosis of subcapsular liver hematoma was made in the antepartum period for six patients and was made in the postpartum for seven patients. The diagnosis was confirmed in all cases by computed tomography. Conservative management with close hemodynamic monitoring and transfusion of blood and blood products was sufficient in 11 (84.6%) patients; two patients underwent surgical exploration. The mean duration of hospital stay was 10 days (range 2-21 days). Maternal complications included pleural effusions, acute kidney injury, and pulmonary edema. There were no maternal deaths. There were four stillbirths and no neonatal deaths. Four people had five subsequent pregnancies; delivery was preterm in all five pregnancies, two pregnancies were complicated by subsequent HELLP syndrome, and one patient developed recurrent subcapsular liver hematoma.Subcapsular liver hematoma is a rare complication of preeclampsia, eclampsia, and HELLP syndrome that is associated with substantial maternal and perinatal morbidities. Conservative management with hemodynamic monitoring and transfusion of blood and blood products was sufficient for management in the majority of cases. All subsequent pregnancies resulted in preterm births.Copyright © 2025 by the American College of Obstetricians and Gynecologists. Published by Wolters Kluwer Health, Inc. All rights reserved.
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Liver function abnormalities are noted in a minority of pregnancies with multiple causes for the same. A small proportion of these develop severe liver injury and progress to acute liver failure (ALF). There is a discrete set of etiology for ALF in pregnancy and comprehensive understanding will help in urgent evaluation. Certain diseases such as acute fatty liver of pregnancy, hemolysis, elevated liver enzyme, low platelet (HELLP) syndrome and pre-eclampsia are secondary to pregnant state and can present as ALF. Quick and targeted evaluation with urgent institution of etiology-specific management, especially urgent delivery in patients with pregnancy-associated liver diseases, is the key to avoiding maternal deaths. Pregnancy, as also the fetal life, imparts a further layer of complication in assessment, prognosis and management of these sick patients with ALF. Optimal management often requires a multidisciplinary approach in a well-equipped centre. In this review, we discuss evaluation, assessment and management of pregnant patients with ALF, focussing on approach to pregnancy-associated liver diseases.© 2024. Indian Society of Gastroenterology.
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A thirty-year-old pregnant woman was admitted to hospital with headache and gastrointestinal discomfort. She developed peripheral oedema and had an emergency caesarean section following an episode of tonic-clonic seizures. Her delivery was further complicated by postpartum haemorrhage and she was admitted to the Intensive Care Unit (ICU) for further resuscitation and seizure control which required infusions of magnesium and multiple anticonvulsants. Despite haemodynamic optimisation she developed an acute kidney injury with evidence of liver damage, thrombocytopenia and haemolysis. Haemolysis, Elevated Liver enzymes and Low Platelets (HELLP) syndrome, a multisystem disease of advanced pregnancy which overlaps with pre-eclampsia, was diagnosed. HELLP syndrome is associated with a range of complications which may require critical care support, including placental abruption and foetal loss, acute kidney injury, microangiopathic haemolytic anaemia, acute liver failure and liver capsule rupture. Definitive treatment of HELLP is delivery of the fetus and in its most severe forms requires admission to the ICU for multiorgan support. Therapeutic strategies in ICU are mainly supportive and include blood pressure control, meticulous fluid balance and possibly escalation to renal replacement therapy, mechanical ventilation, neuroprotection, seizure control, and management of liver failure-related complications. Multidisciplinary input is essential for optimal treatment.
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We report a subgroup of patients with fulminant hemolysis, elevated liver enzymes, low platelet count (HELLP) syndrome, manifesting extreme elevation of aspartate aminotransferase (AST; SGOT) and lactate dehydrogenase (LDH) levels and abnormal mental status. These gravidas are at high risk for mortality. Only four patients treated by the authors over a 10-year period have had AST more than 2000 IU/L and LDH more than 3000 IU/L in the HELLP syndrome. This report is based on retrospective chart review. All patients manifested disordered mental status, jaundice, intense hemolysis, and extreme hypertension. One patient had developed multiple organ system failure, was moribund at initial perinatal consultation, and died. The three others were treated with aggressive afterload reduction and plasma infusion or plasmapheresis; two survived. Fulminant HELLP syndrome occurs rarely, but marks a group of patients at high risk for mortality. Optimal therapy is unclear; early intervention, including afterload reduction, volume expansion, and consideration of plasma infusions or plasmapheresis, is recommended.
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HELLP syndrome, characterized by the triad of hemolysis, elevated liver enzymes due to liver impairment, and low platelet count, is a hypertensive disorder in pregnancy. Although it is said to be caused by disturbed placentation in the first trimester, its clinical presentation can be seen mostly in the third trimester, but never before the completed 20 gestational week. Predictive for its diagnosis is the reported upper abdominal pain that normally is localized under the right arc of ribs. With the aid of laboratory examination, the suspected diagnosis can be confirmed or excluded. Therapeutic options are observational treatment with the prophylaxis of respiratory distress syndrome and attempting to prolong pregnancy with the help of steroids like dexamethasone, or delivering the infant by inducing labor or performing a primary caesarian section, depending on the gestational week. Delivery is the unique causal therapy of HELLP syndrome. Clinical management is mainly influenced by the course of HELLP syndrome. There are mild forms that allow prolonging the pregnancy for several days or sometimes weeks, but also foudroyant courses with acute liver damage. We report the case of a 40-year-old, gravida 1 woman in gestational week 36+1 who was brought to our hospital in hemorrhagic shock caused by a rupture of the liver due to acute HELLP-syndrome.© Georg Thieme Verlag KG Stuttgart · New York.
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杨柳, 邹丽. HELLP综合征诊治的最新认识[J]. 中华产科急救电子杂志, 2021, 10(3):142-146.DOI:10.3877/cma.j.issn.2095-3259.2021.03.004.
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Hemolysis, Elevated Liver Enzymes, Low Platelets syndrome (HELLP syndrome) is one of the actively haunted maternal morbidity through Slovak Obstetric Survey System (SOSS), the organisation for surveillance of severe maternal morbidity and mortality in Slovakia.The questionaires were sent in 55 Obstetric Units in Slovakia. The analyzed and here presented data had been collected cases those happened in the period from 1.1.2012 till 31.12.2014. Controls were women from SR during observed years, who delivered without HELLP syndrome.The return rate of questionnaires was 89.67 %, thus covering 146 972 deliveries during the study period in Slovakia. The exact incidence of HELLP syndrome was 0.63/1000 deliveries (CI 95 % 0.51-0.78). Risk factors were age > 30 (OR = 1.63), nuliparous (OR = 2.96), pregnancy after assisted reproduction technology (OR = 8.29) and multiple pregnancy OR = 9.19). The mean gestation age at delivery was 33.8th weeks. Vaginal delivery was by 10 (10.8 %) patients with HELLP syndrome and in the 83 (89.2 %) patients pregnancy was terminated with acute caesarean section. There were reported 45 050 cases (30.7 %) of the caesarean section in the control group throughout the study period.The older age, nulliparity, multiple pregnancy and pregnancy after assisted reproduction techniques was identified as a significant risk factors of HELLP syndrome (Tab. 5, Fig. 1, Ref. 21).
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HELLP syndrome was first described in 1982 by Weinstein et al. and the term HELLP refers to an acronym used to describe the clinical condition that leads to hemolysis, elevated liver enzymes and low platelets. The syndrome frequency varies from 0.5 to 0.9% pregnancies and manifests preferentially between the 27th and 37th week of gestation. Approximately 30% of cases occur after delivery. Although the etiopathogenesis of this syndrome remains unclear, histopathologic findings in the liver include intravascular fibrin deposits that presumably may lead to hepatic sinusoidal obstruction, intrahepatic vascular congestion, and increased intrahepatic pressure with ensuing hepatic necrosis, intraparenchymal and subcapsular hemorrhage, and eventually capsular rupture. Typical clinical symptoms of HELLP syndrome are pain in the right upper quadrant abdomen or epigastric pain, nausea and vomiting. However, this syndrome can present nonspecific symptoms and the diagnosis may be difficult to be established. Laboratory tests and imaging exams are essential for differential diagnosis with other clinical conditions. Treatment of HELLP syndrome with corticosteroids, targeting both lung maturation of the fetus is still an uncertain clinical value. In conclusion, three decades after the tireless efforts of Dr. Weinstein to characterize HELLP syndrome, it remains a challenge to the scientific community and several questions need to be answered for the benefit of pregnant women.
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The HELLP syndrome (hemolysis, liver damage and thrombocytopenia) is a rare (0.5–0.9%) but serious complication of pregnancy or puerperium associated with a higher risk of maternal and fetal mortality and morbidity. Liver and spleen hematomas rarely entangle (<2%) HELLP cases, but rupture of the hematomas presents an immediate threat to life. We present the history of a 35-year old pregnant woman (at the 31st week) admitted to our hospital due to the risk of premature delivery. On the first day, the patient did not report any complains, and the only abnormality was thrombocytopenia 106 G/L. However, within several hours, tests showed platelet levels of 40.0 G/L, LDH 2862.0 U/L and AST 2051.6 U/L, and the woman was diagnosed with severe HELLP syndrome, complicated by hematomas of the liver and spleen, seizures (eclampsia), severe arterial hypertension and coagulation disorders. The purpose of this article is to highlight the need for early investigation of the causes of thrombocytopenia and the differentiation of HELLP from other thrombotic microangiopathies (TMAs).
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A woman at 16 weeks of gestation was admitted to our perinatal center with unspecific abdominal pain. The results from blood samples 12 h after admission revealed a fulminant HELLP-syndrome. After starting i.v. corticosteroid therapy, the woman recovered quickly. CVS was performed because of abnormal findings by ultrasound and a fetal triploidy (69, XXX) was diagnosed. Pregnancy was terminated and histopathological examination of the placental tissue confirmed a partial mole.
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Postpartum onset of eclampsia and HELLP (hemolysis, elevated liver enzymes, low platelet count) syndrome is a rare but life-threatening complication for both mother and fetus. A case of a 38-year-old parturient (gravida 2, para 1) who was asymptomatic prior to delivery is reported. Emergency caesarean section had to be performed due to sudden onset of fetal bradycardia as a result of partial placental separation. The perioperative course was characterized by new onset hypertension, nausea and restlessness; within 2 h the patient suffered a generalized seizure which was treated with magnesium sulfate and hydralazine. Despite management in accordance with current guidelines, the condition deteriorated with hypotension, anemia and renal failure. On further examination hematomas in the abdominal cavity and walls were identified and laboratory tests confirmed HELLP syndrome with severe coagulopathy. Explorative laparotomy revealed diffuse bleeding without a significant isolated source or postpartum uterine hemorrhage. Retrospectively, the anemia could be ascribed to severe hemolysis and diffuse bleeding from coagulopathy. The patient required packed red cells, platelets, fresh frozen plasma and prothrombin complex. After admission to the intensive care unit persistent diffuse bleeding mainly caused by hyperfibrinolysis and renal failure occurred, which required blood transfusion, antifibrinolytic (tranexamic acid) and renal replacement therapy (continuous veno-venous hemodiafiltration with citrate) for 6 days. The patient recovered without any sequelae and was discharged 26 days later. Placental separation with new onset peripartum hypertension is to be interpreted as a precursor of severe gestosis and associated complications, especially disseminated intravascular coagulation (DIC), acute renal failure and pleural effusion. A differentiation between a rapid drop in hemoglobin concentration secondary to hemolysis in postpartum HELLP syndrome rather than postpartum hemorrhage can be challenging. In addition, HELLP syndrome can lead to rapidly developing, fulminant hyperfibrinolysis in the context of DIC. Keys to successful management of postpartum gestosis and associated complications are early detection and perception of clinical and laboratory warning signs, a multidisciplinary approach with rapid and consistent targeted symptomatic therapy to save the mother and fetus.
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A severe hepatopathy constitutes a serious threat during pregnancy and poses considerable challenges to the treating physicians. A broad spectrum of pregnancy-dependent or independent diseases like HELLP-syndrome, liver infection or acute fatty liver of pregnancy (AFLP) is characterized by these affections of the liver. In this study, we present a series of 3 cases with life-threatening hepatopathies and discuss the current state of the literature. A special focus is placed on pathogenesis and differential diagnosis.Pathological, radiological and gynaecological/surgical procedures were performed according to the current German guidelines. Laboratory tests were conducted in the clinics' routine diagnostics section. The existing literature was reviewed via the US National Library of Medicine database “PubMed.gov”.The first patient had been afflicted by a fulminant HELLP syndrome causing delivery after 32 weeks of pregnancy. Consecutively, she suffered a sub-total liver infarction followed by a severe coagulopathy and septic peritonitis. The second patient was diagnosed with HELLP syndrome at 36 weeks of pregnancy. The initially mild syndrome exacerbated after delivery leading to haemorrhagic shock and acute renal failure. In the third case, a woman with asymptomatic hepatitis B delivered in the 36th week of pregnancy. Post partum, her pre-existing condition worsened fulminantly resulting in sub-acute liver dystrophy and massive coagulopathy.Whenever a hepatopathy occurs during pregnancy, several divergent diagnoses with severe implications and different aetiopathologies have to be considered. Diagnostic and therapeutic strategies have to be weighed quickly to enable a fast, interdisciplinary cooperation in order to prevent fatal outcomes.© Georg Thieme Verlag KG Stuttgart · New York.
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Hepatic infarction is a rare and fatal complication associated with hemolysis, elevated liver enzymes and low platelets syndrome. It can develop into fulminant liver failure and lead to death in 16% of cases. A 25-year-old woman, with unremarkable prenatal history, was sent to gynecological emergency unit for management of severe preeclampsia at 30 weeks and 4 days of pregnancy. Initial laboratory studies revealed aspartate aminotransferase at 290 U/L, alanine aminotransferase at 193 U/L and a normal value of hemoglobin, platelets and the prothrombin time. Behind the persistence of high blood pressure despite dual therapy, an emergent cesarean section was performed. However, two days after surgery, the patient accused an epigastric pain and was subsequently noted to have developed HELLP syndrome: thrombocytopenia (77000 /ul), anemia (hemoglobin 9.1 g/dL) and worsened liver injury (aspartate aminotransferase 2809 U/L; alanine aminotransferase 2502 U/L). A thoraco-abdominopelvic computed tomography (CT) was performed, which revealed massive hepatic infarction more marked on the right lobe, by showing the existence of diffuse hypodense plaques, poorly limited, not enhanced after injection, interesting all hepatic segments. The vascular permeability of the portal and subhepatic was preserved. During the surveillance, the laboratory tests worsened (hemoglobin = 4,6 g/dl; platelets count = 20000 /ul; WBC = 26000 /ul; CRP = 340 mg/l; albumin = 16 g/l, prothrombin time (PT) = 50%). The patient received antibiotics, she was transfused by red blood cells and platelets concentrates, she also received albumin with the pleural effusion drainage. The damaged hepatic areas stayed stable in control CT and the patient gradually improved here biological test, to become normal at 11 days after delivery. Hepatic infarction is an extraordinarily rare complication of preeclampsia. The diagnosis should be suspected by noting elevated liver enzymes, thrombocytopenia and typical images of hepatic infarction on abdominal CT. Early recognition and multidisciplinary management is necessary to prevent hepatic failure and death.© Linda El Allani et al.
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The objective of this study was to evaluate acute liver injury (ALI) detected by diffusion-weighted magnetic resonance imaging (MRI) and the associated laboratory findings in women with hemolysis, elevated liver enzymes, and low platelet (HELLP) syndrome.This was a retrospective, observational study of women with HELLP syndrome defined by serum aspartate aminotransferase (AST) levels ≥100 U/L and thrombocytopenia with platelets ≤100,000/µL. All women underwent MRI postpartum including diffusion-weighted imaging to estimate the volume of ALI with reconstructed apparent diffusion coefficient (ADC) maps. The ADC map and the volume of ALI were compared with laboratory abnormalities by Spearman's correlation analysis.From March 2013 through August 2015, 16 women with HELLP syndrome underwent MRI, and of these, 14 (88%) women had areas of increased signal intensity suggestive of ALI. Their median (range) maximum AST level was 262 (140-1,958) IU/L, and at the time of MRI, AST was 103 (36-1,426) IU/L. Both of these AST levels significantly correlated with ADC map as well as the volume of ALI (both -values <0.001).Women with HELLP syndrome frequently exhibited areas of abnormal diffusion in the liver on diffusion-weighted MRI, suggestive of ALI. The extent of liver injury was significantly correlated with serum AST.Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.
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Unexpected rapid death after delivery due to HELLP syndrome (HS) may become the subject of a forensic expertise. Since this syndrome is rarely encountered in forensic pathology, our objective was to point to some specific findings which might present forensic aspects of HS. These include unexpectedness, suddenness and fulminant course of this syndrome, which may confuse physicians, and on the other hand these characteristics cast doubt on violent injury, diagnostic oversights or iatrogenic injuries. Absence of classical signs of preeclampsia and non-specific clinical symptoms cause considerable differential diagnostic problems leading to a diagnostic delay or initial wrong non-obstetric diagnosis. A definitive postmortem diagnosis of HS in questionable cases of maternal death and consecutive forensic expertise of suspected medical malpractice should be based on accepted laboratory criteria and characteristic histopathological alterations.
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Crimean–Congo hemorrhagic fever (CCHF) is fatal in 10 to 40% of cases. It is caused by CCHF virus (CCHFV). Symptoms include fever, headache, myalgia, and often hemorrhage and other complications. This report shows that CCHF may resemble HELLP syndrome (hemolysis, elevated liver enzymes, low platelets). We report CCHF in a pregnant mother with fever and suspected HELLP syndrome, who survived, and her infant (week 36), who died six days after C-section. The high CCHF viral load and bacterial sepsis may have jointly contributed to the death of the infant. CCHF should be considered as a differential diagnosis of HELLP syndrome in regions where this viral disease is endemic.
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Wilson's disease (WD) is an autosomal recessive disorder. It is characterized by toxic accumulation of copper mainly in the liver and brain but also in cornea and kidney due to a defect in biliary excretion of copper. The hepatic manifestation of WD is diverse and may include asymptomatic elevation of aminotransferase, chronic hepatitis, cirrhosis, or acute/fulminant hepatic failure. Characteristic of acute hepatic failure in WD is concomitance of acute intravascular hemolytic anemia. Acute intravascular hemolytic anemia and thrombocytopenia in WD may be interpreted as a feature of Hemolysis, Elevated Liver Enzymes, Low Platelet Count (HELLP) syndrome besides acute liver failure. The differential diagnosis may be very difficult. Here, WD in pregnancy presenting with clinical symptoms of HELLP syndrome and developing acute liver failure in postpartum period is discussed.
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