Background: HELLP syndrome is a rare disease in China, and 20% of patients with severe preeclampsia have been accompanied with HELLP syndrome, which is characterized by the presence of hemolysis, elevated liver enzymes and low platelet count. Case presentation: In this case, we reported that a patient with preeclampsia was diagnosed with HELLP syndrome. Furthermore, hepatic infarction also was found via the computed tomographic (CT) images, which showed peripheral wedge-shaped inhomogeneous low attenuation in the right hepatic lobes via plain CT scan, and the low-density shadow and mottled appearance in the same areas where vessels were seen coursing through them via contrast-enhanced CT scan. Conclusions: Besides typical clinical manifestations of the pregnant patient with preeclampsia, the typical laboratory evidences were elevated liver enzymes and thrombocytopenia. The abdominal CT scan showed imaging features of hepatic infarction, which was helpful to identify the rare complication of HELLP syndrome. Thus, we diagnosed a patient with HELLP syndrome with hepatic infarction, though the patient had no chance to do the liver biopsy. Keywords: Preeclampsia, HELLP syndrome, Hepatic infarct Background differential diagnosis. Once a clinical diagnosis of Preeclampsia (PE) is a pregnancy specific disorder which HELLP syndrome has been confirmed, it need aggressive is characterized by new onset hypertension and protein- interventions with control blood pressure, anti-seizure uria after the 20th weeks of gestation [1, 2]. In the most prophylaxis, corticosteroid treatment for fetal lung ma- severe form of preeclampsia, the presence of HELLP turity enhancement, and expeditious delivery . This syndrome (defined as: hemolysis, elevated liver enzymes case was collected in the First Affiliated Hospital of Xi’an and low platelet) with the addition of hepatic comprom- Jiaotong University in China. ise , such as hepatic infarction or hematoma [4, 5], increased the risk of maternal morbidity and mortality . Case presentation HELLP syndrome should be carefully distinguished A 31-year-old pregnant patient, with a history of two preg- with the diseases, such as benign thrombocytopenia of nancy losses at first half of pregnancy and no history of pregnancy and virus hepatitis. It’s very important to autoimmune or thromboembolic diseases, was evaluated at identify the diagnosis of HELLP syndrome from other 33 + 4 weeks of gestation. An episode of symptomatic renal lithiasis requiring placement of double J stent at 3rd week of gestation was reported by the patient at admission. * Correspondence: firstname.lastname@example.org; email@example.com † The patient was admitted in local hospital due to Qinyue Guo and Zhengfei Yang contributed equally to this work. Department of Respiratory Medicine, the First Affiliated Hospital of Xi’an dizziness, headache and blurred version,and worsening Jiaotong University, Xi’an, China lower extremity edema 10 days ago. Examination results Department of Critical Care Medicine, the First Affiliated Hospital of Xi’an showed blood pressure 170/100 mmHg, proteinuria 3+ Jiaotong University, 277 Yanta West Road, Xi’an 710061, Shaanxi, China Full list of author information is available at the end of the article and occult blood 3+ by urinalysis. Immediate caesarean © The Author(s). 2018 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Guo et al. BMC Pregnancy and Childbirth (2018) 18:191 Page 2 of 4 section was taken as diagnosed with severe preeclamp- contrast-enhanced CT scan (Fig. 1b). Base on CT images, sia. After the surgery, laboratory tests displayed elevated liver infarction was diagnosed. Further tests showed that liver enzyme levels: peak aspartate transaminase (AST) amylase in plasma and urine was negative, and hepatitis B and alanine transaminase (ALT) (1758 u/L and 2158 u/L antigens and antibody, and hepatitis A antibody were all respectively), decreased platelet count (20 × 10 /L) and negative, and the levels of glucose and blood ammonia were hemoglobin value (65 g/L), increased white blood cells normal. Therefore, we further confirmed the diagnosis (WBC) (27.45 × 10 /L) and serum creatinine (233 umol/L) HELLP syndrome with liver infarction. and urea (31.6 mmol/L). Symptomatic treatments were Then we continued treating the patient with antibi- ineffective, so the patient was transferred to our hospital otics, CRRT, drainage of pleural effusion and ascites, and with the primary diagnosis of HELLP syndrome and the supportive treatments. Intravenous dexamethasone severe preeclampsia. 10 mg/d for 3 days was initiated. The results of labora- On admission, the condition of the patient had worsened, tory tests were normalized within 17 days. The damaged and the heart rate was 120 beats per minute, the blood areas were recovered on 17th days after admission via pressure was 138/74 mmHg (controlled by nitroprusside), the second enhanced CT (Fig. 1c and d). She remained and the temperature was 36.5 °C. The pitting ankle edema, asymptomatic and had no complications during headache and blurred version had gradually worsened 6 months of follow-up. accompanied with abdominal distension and weak chest, and decreased urine output. On physical examination, she Discussion and conclusions appeared to be acutely ill with decreased breath sounds, the In this case, we reported that a pregnant woman with results of laboratory tests were not significantly improved preeclampsia was diagnosed with HELLP syndrome than before, accompanied with negative urobilinogen, total (Hemolysis, increased liver enzyme, and low platelets bilirubin 25.5umol/L and reduced serum albumin (Table 1). count). Furthermore, hepatic infarction also found via The ultrasonic images of hepatic parenchyma were abnor- the computed tomographic (CT) images, which showed mal, which may result from blood supply deficiency, and peripheral wedge-shaped inhomogeneous areas of low massive hydrothorax and ascites. Based on all above, we di- attenuation in the right hepatic lobes via plain CT scan, agnosed the patient as HELLP syndrome, but could not and the low-density shadow and mottled appearance in fully rule out other diseases with similar symptoms. The the same areas where vessels were seen coursing therapy including continuous renal replacement therapy through them via contrast-enhanced CT scan. Thus, the (CRRT),plasmaexchangeand othersupportivetreatments case of HELLP syndrome is rare to be found accompan- such as red cell transfusion, platelet concentrates transfu- ied with liver infarction. Abdominal CT is often helpful sions and antihypertensive were provided for the patient for the management of HELLP syndrome to rule out immediately. hepatic hematoma, and in this case, we found that liver Three days after admission to our hospital, plain CT scan infarction was induced by HELLP syndrome. showed peripheral wedge-shaped inhomogeneous low at- Liver infarction is a very rare event because of hepatic tenuation in the right hepatic lobes (Fig. 1a), and the low- distinct double blood supply. Most cases of liver infarc- density shadow and mottled appearance in the same areas tion result from interference with arterial blood supply where vessels were seen coursing through them via to the liver, due to pregnancy-induced hypertension Table 1 laboratory and blood chemical findings Local hospital 3th hospital day 17th hospital day Glucose Normal Normal Normal Proteinuria 3+ 3+ Normal Aspartate transaminase (AST)(U/I) 1758 85 12 Alanine transaminase (ALT)(U/I) 2158 98 10 Total bilirubin (umol/L) 27.5 24 12 White blood cells (WBC)(*10 /L) 27.5 32.6 3.7 Hemoglobin (g/L) 65 65 81 Platelet count (*10 /L) 20 26 155 Serum creatinine (umol/L) 233 135 81 Urea (mmol/L) 31.6 11.6 3.6 Cholesterol(mmol/L) 4.09 2.99 3.05 Triglycerides(mmol/L) 3.31 2.01 1.70 Guo et al. BMC Pregnancy and Childbirth (2018) 18:191 Page 3 of 4 Fig. 1 a Non-contras CT demonstrates peripheral wedge-shaped inhomogeneous low attenuation in the right hepatic lobes on 3th after admission. b Contrast-enhanced CT of the liver shows mottled appearance in the same areas where vessels were seen coursing through. c and d showed the damaged areas were reduced in the enhanced CT 17th days after admission (PIH), anti-phospholipid syndrome (APS), ischemic creatinine and urea all supported the criteria for both hepatitis, and portal vein thrombosis (PVT) and so on PE and HELLP syndrome , but the pathogenesis [8, 9]. Clinical manifestations of liver infarction are non- of both PE and HELLP is still unknown. Fortunately, specificity, suddenly upper abdominal pain, fever, jaun- CRRT and plasma exchange were effective for the dice, and suddenly increased liver aminotransferases. patient, and after hormone therapy and supportive The liver biopsy is helpful to distinguish liver infarction treatments, the damaged areas of liver were recovered from other diseases such as liver abscess or cholangio- and the patient got better. carcinoma, while, imaging examination also play an im- Liver infarction is rare with the consequences of portant role in diagnosis. Although hepatic infarction is HELLP syndrome . CT scan may be a useful method nonspecific and may be caused by a variety of diseases, for the differential diagnosis, which showed peripherally the CT images of the liver appear to be helpful for the wedge-shaped inhomogeneous areas of low attenuation differential diagnosis of liver dysfunction. A few cases of with enhanced vessels coursing through these areas. liver infarction induced by HELLP syndrome have been Based on the clinical manifestation, the CT images, and reported in China . other laboratory tests (For example the levels of glucose HELLP syndrome happened in about 20% of patients and blood ammonia are normal), we could rule out with severe preeclampsia . The diagnosis of HELLP other diagnoses and make a definite diagnosis for syndrome could be made through typical results of HELLP syndrome with liver infarction. laboratory tests, including signs of hemolytic anemia In conclusion, we reported a patient diagnosed with and thrombocytopenia with platelets < 100,000 cells/ul, the HELLP syndrome with hepatic infarction. The elevated AST, ALT and lactate dehydrogenase, associ- clinical manifestations of the pregnant patient with pre- ated with clinical manifestation of right upper quadrant eclampsia were headache, blurred version, abdominal pain, nausea, vomiting, malaise, headache and edema, distension and weak chest. The typical laboratory evi- but some women with HELLP syndrome may be dences were elevated liver enzymes, thrombocytopenia. asymptomatic. Thus, we diagnosed the patient with the HELLP syn- In this case, proteinuria, pregnancy-induced hyperten- drome. The abdominal CT scan showed imaging features sion, edema, elevated liver enzyme levels, decreased of hepatic infarction, which was helpful to identify the platelet count and hemoglobin, rapidly increased serum rare complication of HELLP syndrome. Guo et al. BMC Pregnancy and Childbirth (2018) 18:191 Page 4 of 4 Abbreviations 8. Zissin R, Yaffe D, Fejgin M, et al. Hepatic infarction in preeclampsia as part AFLP: Acute fatty liver of pregnancy; ALT: Alanine transaminase; APS: Anti- of the HELLP syndrome: CT appearance. Abdom Imaging. 1999;24(6):594–6. phospholipid syndrome; AST: Aspartate transaminase; CRRT: Continuous renal 9. Miyakoshi K, Tanaka M, Ono A, et al. Massive hepatic infarction in replacement therapy; CT: Computed tomographic; DIC: Disseminated intravascular preeclampsia: successful treatment with continuous hemodiafiltration and coagulation; PE: Preeclampsia; PIH: Pregnancy-induced hypertension; corticosteroid therapy. J Perinat Med. 2004;32(5):453. PVT,: Ischemic hepatitis, portal vein thrombosis; WBC: White blood cells 10. Einar S, Kjell H, Ulrich A. The HELLP syndrome: clinical issues and management. A review. BMC Pregnancy Childbirth. 2009;9(1):8. 11. Kronthal AJ, Fishman EK, Kuhlman JE, et al. Hepatic infarction in Funding preeclampsia. Radiology. 1990;177(177):726–8. This work was supported by the programs Young Innovators Awards of the 12. Ibdah JA. Acute fatty liver of pregnancy: an update on pathogenesis and First Affiliated Hospital of Xi’an Jiaotong University (2015YK6) in the design of clinical implications. World J Gastroenterol. 2007;12(46):7397–404. the study and collection, and Natural Science Foundation of Shaanxi 13. Holbert BL, Baron RL, Dodd GD 3rd. Hepatic infarction caused by arterial province (2017JM8016) in analysis and interpretation of data. insufficiency: spectrum and evolution of CT findings. Am J Roentgenol. 2013;166(4):815–20. Availability of data and materials All data generated or analysed during this study are included in this published article. Authors’ contributions ZY, JG, LZ and LG analyzed and interpreted the patient data. QG contributed to collection of data and writing of the manuscript. BZ contributed to support of funding and revision of manuscript. QS contributed to supervision of the research group and revision of manuscript. All authors read and approved the final manuscript. Ethics approval and consent to participate All procedures were performed in accordance with the guidelines in the Declaration of Helsinki, and were approved by the ethics committee, the First Affiliated Hospital of Xi’an Jiaotong University. The patient was treated solely according to standard treatment. Consent for publication Written informed consent was obtained from the patient for the publication of this case report and any accompanying images. Competing interests The authors declare that they have no competing interests. Publisher’sNote Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. Author details Department of Critical Care Medicine, the First Affiliated Hospital of Xi’an Jiaotong University, 277 Yanta West Road, Xi’an 710061, Shaanxi, China. Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China. Department of Hepatobiliary Surgery, the First Affiliated Hospital of Xi’an Jiaotong University, Xi’an, China. Department of Respiratory Medicine, the First Affiliated Hospital of Xi’an Jiaotong University, Xi’an, China. Received: 23 January 2017 Accepted: 30 April 2018 References 1. Sibai B, Dekker G, Kupferminc M. Pre-eclampsia. Lancet. 2005;365(9461):785. 2. Brown MA, et al. The classification and diagnosis of the hypertensive disorders of pregnancy: statement from the International Society for the Study of hypertension in pregnancy (ISSHP). Hypertens Pregnancy. 2001; 20(1):IX–XIV. 3. Sibai BM. Diagnosis, controversies, and management of the syndrome of hemolysis, elevated liver enzymes, and low platelet count. Obstet Gynecol. 2004;103(1):981–91. 4. Hay JE. Liver disease in pregnancy. Hepatology. 2008;47(3):1067–76. 5. Tran TT, Ahn J, Reau NS. ACG clinical guideline: liver disease and pregnancy. Am J Gastroenterol. 2016;111(2):176–94. 6. Haddad B, et al. Risk factors for adverse maternal outcomes among women with HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome. Am J Obstet Gynecol. 2000;183(2):444–8. 7. Witlin AG, Saade GR, Mattar F, Sibai BM. Risk factors for abruption placentae and eclampsia: analysis of 445 consecutively managed women with severe preeclampsia and eclampsia. Am J Obstet Gynecol. 1999;180:1322–9.
BMC Pregnancy and Childbirth – Springer Journals
Published: May 30, 2018
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