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HELLP syndrome--a case study.


HELLP syndrome was first described by Pritchard et al in 1954 (1). However, the first published article naming the syndrome as HELLP appeared in the literature almost thirty years later. Weinstein reported his findings in a unique group of pre-eclamptic/eclamptic women who had a severe form of pre-eclampsia characterised by haemolysis, elevated liver enzymes, and a low platelet count (2). He devised the acronym HELLP to name the syndrome. HELLP syndrome occurs in 4-12% of women with severe pre-eclampsia and can manifest at any stage during pregnancy. Approximately 30% of cases occur postpartum and in these cases, only 80% will have been diagnosed as pre-eclampsia antenatally (3).

HELLP syndrome can be associated with poor maternal and perinatal outcome. Maternal morbidity is mostly associated with disseminated intravascular coagulation (DIC), placental abruption, acute renal failure and ruptured liver haematoma. Most perinatal deaths are associated with placental abruption, intrauterine asphyxia and prematurity.

Case study

The patient had recently had a normal vaginal delivery of twins at 36+6 weeks gestation on the 9th January 2008 at 0900 and 0918 respectively. Her obstetric history includes two normal vaginal deliveries with no antenatal or postnatal complications. She was recovering in the postnatal ward and as she had exhibited mild symptoms of pre-eclampsia in the weeks leading up to delivery, her vital signs were being closely monitored. The majority of women with pre-eclampsia are expected to recover post delivery, but this patient's condition deteriorated rapidly over the next 24 hours of the postnatal period. Oral Augmentin was prescribed post delivery for 5 days. See tabulated results at the end of the case study.

9 January 2008

1500 Blood pressure had risen from 140/75 to 167/92.

1845 Blood pressure 161/95. Patient complained of chest pain radiating into her back, she vomited and had epigastric pain.

1930 Blood pressure 192/96. Labetalol prescribed 8 hourly. Patient was pale and continued to complain of epigastric tenderness. The clinical team queried whether she was having a reaction to oral Augmentin. Dexamethasone was added to patient's medications.

2020 A significant difference in haematology and biochemistry results was noted as follows:

2050 Blood pressure 192/106.

2100 Marked haematuria was evident. Epigastric pain was worsening. Her consultant gynaecologist was called and due to her thrombocytopaenia, raised liver enzymes and epigastric pain, a diagnosis of HELLP syndrome was made. Her haematuria worsened and she had exaggerated reflexes. Blood pressure was now to be monitored every 15 minutes. ICU specialist consulted and a decision was made to repeat blood tests in 2 hours and if the results had deteriorated further, she was to be admitted to ICU. MgS[O.sub.4] infusion commenced as per ICU protocol. MgS[O.sub.4] minimises the risk of seizures in patients with marked hypertension.

2130 Indwelling catheter was draining frank haematuria. The patient described her chest pain on a 1-10 scale as 1/10. Chest x-ray was performed. Obstetrician was not convinced of the diagnosis of HELLP syndrome as 02 saturation was normal.

2155 Further blood tests taken. In this two hour period the platelet count had dropped even more to 74 x [10.sup.9] ; WBC had risen to 13.0 x [10.sup.9]; AST had risen significantly to 1083 U/L; ALT had risen to 439 x [10.sup.9] and the LDH ws extremely high at 2,386 U/L; GGT was 11 U/L.

The patient now had severe HELLP syndrome. An acute admission from obstetrics to ICU was arranged.

2200 Epigastric pain was now described by the patient as 5/10. Patient declined analgesia.

2300 Patient admitted to ICU. There was a delay in admission to ICU because the obstetrician was not convinced of the diagnosis of HELLP syndrome. Also the biochemistry specimens collected at 2155 were clotted and no biochemistry results were available until 2300.

10 January 2008

0015 Further blood tests taken. The platelet count had dropped even more to 40 x [10.sup.9]; AST had risen higher to 1,482 U/L; ALT was essentially unchanged at 416 U/L; LDH had risen more to 2,585 U/L and GGT was 13 U/L.

0040 Haematologist consulted and it was decided to keep the platelet level at 50 x [10.sup.9] and authorisation to transfuse 1 unit of apheresis platelets was given. Obstetrician also discussed the benefits of plasmapheresis/exchange transfusion with the haematologist. The decision was made that at this stage there was not enough evidence to support this procedure and that this would be reviewed again later in the morning. Target blood pressure was 170/110 or less and controlled with medications.

0115 Blood pressure 192/101. MgS[O.sub.4] infusion increased as per ICU protocol. One off dose of hydrocortisone was administered.

0200 Further blood tests taken. Post platelet transfusion the platelet count had only risen slightly to 50 x [10.sup.9].

0600 Blood pressure 165/84. Further blood tests were taken to monitor the platelet count. Platelet count had now dropped back to 45 x [10.sup.9]. Haematologist decided to keep the platelet level >20 x [10.sup.9].

0740 Further blood tests were taken to monitor the platelet count. Platelet count had now dropped to 40 x [10.sup.9]. A GGT was performed which had now risen slightly to 14 U/L.

1000 Patient was improving clinically. Urine was clearer with excellent urine output. No epigastric pain.

1400 Further blood tests were taken to monitor the platelet count which now was 41 x [10.sup.9].

Further blood tests throughout the day showed no significant differences from the above results.

11 January 2008

0200 Blood pressure 130/70. Urine now clear with no signs of haematuria. Platelet count 44 x [10.sup.9].

0740 Further blood tests taken. Platelet count 45 x [10.sup.9]; AST had fallen significantly to 208 U/L; ALT had dropped to 172 x [10.sup.9] and GGT still was 14 x [10.sup.9].

1030 Transferred from ICU back to obstetric ward.

1100 Blood pressure 131/74.

1400 Blood pressure 120/64.

12-14 January 2008

Final summary of blood tests taken 12-14 January are included in the tabulated results below. The only thing of note was that the GGT kept rising until discharge.




The actual cause of HELLP syndrome is not well understood, however evidence now suggests endothelial cell damage during implantation in the uterine wall is an early stage of the disease (4). Tissue damage and insufficient blood supply due to blocked arteries can develop later in pregnancy in HELLP syndrome. This can cause the obstruction of blood flow and liver distension that can lead to rupture and haemorrhage. Liver distension causes the epigastric or right upper quadrant pain associated with HELLP syndrome.

The presentation of HELLP syndrome may be non-specific and misdiagnosis is more likely when the condition develops before term. Diagnosis may be delayed with consequent risk for mother and fetus (4). The symptoms of HELLP syndrome include epigastric pain, nausea and/or vomiting, non-specific viral illness type symptoms, visual disturbances, headache, bleeding from the gums, jaundice, and neck or shoulder pain. As the classic symptoms of pre-eclampsia such as hypertension and proteinuria are not always present in women with HELLP syndrome, a non-obstetric diagnosis, such as gall bladder disease, viral hepatitis, gastroenteritis, kidney stones, peptic ulcer, acute fatty liver of pregnancy, idiopathic thrombocytopenia purpura, thrombotic thrombocytopenia purpura, pyelonephritis and haemolytic uraemic syndrome, may be made. However, disseminated intravascular coagulation (DIC), placental abruption and fetal death contribute to the significant maternal and fetal morbidity and mortality involved with the condition (3).

The laboratory criteria for the diagnosis of HELLP syndrome most commonly used in clinical practice were defined by Sibai (4). However, there remains confusion regarding terminology and diagnosis and a lack of consensus regarding which tests and levels should be used to diagnose the syndrome. The following presents an overview of laboratory investigations most commonly used in the diagnosis of HELLP syndrome.


Haemolysis, defined as the presence of microangiopathic haemolytic anaemia, is a hallmark of HELLP (5). It has been noted that only a small number of cases have overt haemolysis (5). Haemolysis is confirmed by an abnormal peripheral blood smear with the presence of burr cells, shistocytes and polychromasia. Activation of the coagulation cascade leads to fibrin forming cross-linked networks in the small blood vessels. The red blood cells become damaged when passing through these blood vessels leading to a microangiopathic haemolytic anaemia (4,5).

Elevated Liver enzymes and liver damage

Elevated levels of liver enzymes reflect damage within the liver. Hepatic damage results from micro-emboli in the hepatic vasculature. Jaundice may be present; serum bilirubin levels rise as a result of haemolysis (6). High levels of alanine transaminase (ALT) are specific for hepatic damage (6). Aspartate transaminase (AST) levels increase with liver damage but the enzyme is also found in other organs thus elevated levels of AST are not specific for hepatic damage (6). Gamma glutamyl transpeptidase (GGT) is found almost entirely in the liver and levels of this enzyme are elevated in HELLP syndrome. Lactate dehydrogenase (LDH) is another liver enzyme which is found in other parts of the body but may also be elevated in HELLP syndrome (6). In our case study the ALT, AST and GGT all continued to rise in the post-partum period. These liver enzymes remained raised during the acute phase of the patient's condition and rapidly returned to normal as she recovered.

Alkaline phosphatase (ALP) is another liver enzyme which may be raised in HELLP syndrome and is often still raised after other liver enzymes have returned to normal. Biochemistry results showed that the patient's ALP was 131 U/L two days post delivery, and 2 days later had further increased to 435 U/L. ALP may also be raised due to placental origin rather than liver damage, but the results indicate that in our case study the raised ALP was due to liver damage and not of placental origin.

Tissue damage and ischaemia within the liver leads to obstruction of blood flow and liver distension which potentiates liver rupture (2). The classic hepatic lesion associated with HELLP syndrome is periportal or focal parenchymal necrosis. Pain is usually localised to the right upper quadrant or mid-epigastric region and is caused by distension of the liver. A radiological finding from an abdominal CT scan on the patient showed tiny subcapsular bleeding in the liver. No bleeding point could be identified.

Low Platelets

Platelet consumption occurs in pre-eclampsia as arteriolar vasospasm damages the endothelial layer of small vasculature, forming lesions. The lesions allow platelet aggregation and the formation of a fibrin network (6). In HELLP syndrome the circulating volume of platelets reduces as consumption increases, resulting in thrombocytopenia. A platelet count of <100 x [10.sup.9]/L is significant in the diagnosis of HELLP syndrome (6). Rapid diagnosis and treatment of this patient prevented DIC, a very severe complication of HELLP syndrome. Hydrocortisone and platelet transfusion maintained the platelet count close to 50 x [10.sup.9]. After this critical time, once the threat of DIC had lessened significantly and the patient stabilised further, the platelet count was kept at >20 x [10.sup.9], in keeping with best practice. Keeping the platelet count stable prevented ongoing coagulopathies culminating in DIC.

The syndrome can by typed into Type 1, Type 2 or Type 3 depending on the platelet count with the severe forms generally characterised as Type 1 (4). Women who manifest one or two but not all three of the components of HELLP syndrome have a better prognosis than women with complete HELLP syndrome (4,7). However, it must be realised that as with pre-eclampsia, the natural course of the disease is to worsen over time (5).

The only definitive treatment for women who have HELLP syndrome is delivery. The condition is potentially fatal for mother and fetus with the most common cause of maternal death being liver rupture (8). Deteriorating liver function and/or progressive thrombocytopenia is an indication for delivery, regardless of gestation. It is important to be vigilant as HELLP may develop postpartum.

Several medications have been investigated for the treatment of HELLP syndrome, but with conflicting evidence. A low platelet count is treated with platelet transfusion, DIC with fresh frozen plasma, and anaemia may require transfusion of red cells. In mild cases, corticosteroids and anti-hypertensive medication may be sufficient. Intravenous fluids are generally required.

In conclusion, this case demonstrates the importance of rapid and early diagnosis of HELLP syndrome and treatment of the symptoms to ensure a favourable maternal and perinatal outcome.


(1.) Pritchard JA, Weisman R, Ratnoff OD. Intravascular hemolysis, thrombocytopenia and other hematologic abnormalities associated with severe toxaemia of pregnancy. N Eng Med J 1954; 250: 89-98.

(2.) 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: 159-67.

(3.) Hayman R Myers J. Diagnosis and clinical presentation. In: PE Baker & JCP Kingdom (Eds.). Pre-eclampsia. Current Perspectives on Management. Parthenon Publishing Group, London, 2004: 133-43.

(4.) Sibai BM. The HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets): much ado about nothing? Am J Obstet Gynaecol 1990; 162: 311-6.

(5.) Baxter JK Weinstein L. HELLP syndrome: the state of the art. Obstet Gynecol Surv 2004; 59: 838-45.

(6.) Cowan J. Women's Experience of Severe Early Onset Preeclampsia: A Hermeneutic Analysis. Masters Thesis, AUT, Auckland, New Zealand, 2005.

(7.) Audibert F, Friedman SA, Frangieh AY. Clinical utility of strict diagnostic criteria for the HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome. Am J Obstet Gynecol 1996; 175: 460-4.

(8.) Poole JH. Getting Perspective on HELLP Syndrome. Am J Matern Child Nurs 1998; 13: 432-7.

Robyn Barnett, Medical Laboratory Scientist

Bronwyn Kendrick, Medical Laboratory Scientist

Department of Transfusion Medicine, New Zealand Blood Service Manawatu, Palmerston North

Address for correspondence: Robyn Barnett and Bronwyn Kendrick, Transfusion Medicine Department, NZ Blood Service, Palmerston North.

Email: Email: bronwyn.kendrick@
(reference range)                 0507    1940

Platelets [10.sup.9] (150-400)    138     118
WBC [10.sup.9] (4-10)             7.4     10.5
AST U/L (0-30)                    26      216
ALT U/L (0-40)                    15      105
LDH U/L (125-243)
GGT U/L (0-36)                            9

Table 1. Overview of physiological and pathology results

                      Blood             Platelet
  Date      Time    Pressure     WCC     Count      AST     ALT

 9.1.08     0507                 7.4      138       26      15
            0918     140/75
            1500     167/92
            1845     161/95
            1930     192/96

            1940                10.5      118       216     105
            2050     192/106
            2155                 13        74      1083     439
 10.1.08    0015                           40      1482     416

            0115     192/101

            0200                           50
            0600     165/84                45
            0740                           40
            1400                           41
 11.1.08    0200     130/70                44
            0740                           45       208     172
            1100     131/74
            1400     120/64
 12.1.08    0800                11.6       82       83      116
 13.1.08    0945                 8.2      121       72      99
 14.1.08    0630                 8.0      138       61      91
            2213                14.0      206       44      82
 16.1.08    1515                 8.7      272       20      45

  Date      Time     LDH     GGT    Haematuria     Medications

 9.1.08     0507
            0918                                    Augmentin
            1930                                  Labetalol and
            1940              9
            2100                      marked      MgS[O.sub.4]
            2130                      frank
            2155    2386     11
 10.1.08    0015    2585     13

            0115                                  MgS[O.sub.4]
            0740             14
            1000                      slight
 11.1.08    0200                      Clear
            0740             14
 12.1.08    0800
 13.1.08    0945             100
 14.1.08    0630             121
            2213             135
 16.1.08    1515

  Date      Time     Products

 9.1.08     0507

 10.1.08    0015
            0040     Platelet

 11.1.08    0200
 12.1.08    0800
 13.1.08    0945
 14.1.08    0630
 16.1.08    1515
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Author:Barnett, Robyn; Kendrick, Bronwyn
Publication:New Zealand Journal of Medical Laboratory Science
Article Type:Case study
Geographic Code:8NEWZ
Date:Apr 1, 2010
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