246 SAMT DEEL 67 16 FEBRUARIE 1985
The HELLP (haemolysis, elevated liver
enzymes, low platelet count) syndrome in
severe hypertensive crises of pregnancy
- does it exist?
M. PILLAY, J. MOODLEY
haematological investigations once they had recovered suffi-
cienrly from the acute evenr. The haematocrit, the platelet
counr, a peripheral blood smear and liver function values were
The HELLP syndrome, comprising haemolysis, ele- evaluated on admission (day I) and on day 7 after delivery in
vated liver enzyme values and a low platelet count, all the women in the study.
was studied in 26 women with hypertensive crises of Data were analysed by the paired [-test. Statistical differences
pregnancy and in matched controls. Platelet counts were considered significant if P < 0,05.
and bilirubin, lactate dehydrogenase, aspartate trans-
aminase a[1d haematocrit levels together with peri-
pheral blood smears were studied on the day of Results
admission and on the 7th day after delivery. Only 1
woman with pre-eclampsia was found to have had The clinical characteristics of patients are shown in Table I.
the HELLP syndrome; the case report is presented The maternal and gestational ages were similar in both groups.
and the laboratory investigations are discussed. The mean birth weight -of babies born to mothers in the
reference group was significantly greater than that of those
S Atr Med J 1985: 67: 246 - 248.
born to mothers in the study group.
TABLE I. CLINICAL CHARACTERISTICS OF THE CONTROL
AND STUDY GROUPS (MEAN ± SO)
Weinstein l described a unique entity in women with severe
pre-eclampsia and eclampsia which he termed the HELLP Control group Study group
syndrome. It is characterized by haemolysis, elevated liver (N= 26) (N= 26)
enzyme levels and a low platelet count. In a retrospective Maternal age (yrs)* 26,9 ± 8,60 26,9 ± 8,62
analysis over a 30-month period, he found 29 women who (16 - 45) (15 - 44)
fulfilled the criteria for this syndrome. Because our clinical Gestational age (wks)* 34,2 ± 1,3 34,4 ± 1,5
experience at the King Edward VIII Hospital, Durban, is that (28 - 40) (28 - 40)
this enrity is uncommon, a study was undertaken of women Birth weight (g)t 2400 ± 800 2000 ± 800
with severe hypertensive crises in pregnancy. (500 - 3 400) (500 - 3 400)
,. 0 statistical difference.
'P = 0.0097.
Ranges are given in parentheses.
Patients and methods
A prospective study of 26 women with severe pre-eclampsial
eclampsia was undertaken over a 6-month period (February- In the study group there were 17 primigravidas and 9
August 1984). Severe pre-eclampsia was defined as a blood multiparas. There were 3 eclamptic patients in the multiparous
pressure of 160/110 mmHg or higher accompanied by genera- group (33%) and 13 (76%) in the primiparous group.
lized oedema and proteinuria of at least ++ on Dipstix Table 11 shows the haematological findings in both groups
examination. Eclampsia was defined as convulsions associated on day I and day 7. There was no significant difference
with severe hypertension. Management of these conditions is between the groups as regards mean haematocrit levels and
described elsewhere. 2 platelet counts. An abnormal red cell morphological appear-
The control group consisted of 26 normotensive gravid ance on the peripheral smear, compatible with a diagnosis of
women mat,ehed for maternal age, parity and gestational age micro-angiopathic haemolytic anaemia, was noted in I woman
and with no medical complications. Informed consent was with severe hypertension; she was the only woman to manifest
obtained from both groups; the women with a diagnosis of evidence of the classic HELLP syndrome, and her case report
eclampsia were informed of the necessity for extensive appears below. There was no significant difference in liver
function test results between the groups either on day I or on
day 7 (Table Ill).
Department of Obstetrics and Gynaecology, University of
Natal and King Edward VIII Hospital, Durban
M. PILLAY, .\I.B. CH.B.,Registrar Case report
J. MOODLEY, M.B. CH.B., F.C.O.G. (SA), Consulcanc
A 33-year-old primigravid black patient presented to the
obstetric unit at King Edward VIII Hospital with severe
Reprint requests [0: Or ]. Moodley, Dept of Obstetrics and Gynaecology, Univer ity of
:-\alal, PO Box 17039, CongcUa, 4013 RSA. pregnancy-induced hypertension associated with severe genera-
SAMJ VOLUME 67 16 FEBRUARY 1985 247
TABLE 11. MATERNAL HAEMATOLOGICAL FINDINGS (MEAN ± SO)
Day 1 Day 7
Controls Study group Controls Study group
(N=26) (N = 26) (N = 26) (N= 26)
Haematocrit (%)* 35,3±3,6 35,O±4,7 34,1 ±4,5 35,O±4,7
Platelet count (x 10 9 /1)* 191,5±40,1 190,8 ± 54,7 193,3 ± 39,8 201,0 ± 50,3
MAHA o 1 0 0
• 0 statIstical difference.
MAHA = micro-anglopathlc haemolytic' anaemIa.
TABLE Ill. ANALYSIS OF LIVER FUNCTION TEST RESULTS (MEAN ± SO)
Day 1 Day 7
Controls Study group Controls Study group
Bilirubin (}lmol/l) 9,158±4,33 11,63 ± 4,29 8,52 ± 3,09 9,78 ± 3,34
LD (U/I) 202,2 ± 47,51 219,5 ± 57,69 203,52 ± 41,05 211,00 ± 54,95
AST (U/I) 13,30±3,62 15,88 ± 5,21 14,00 ± 3,62 15,27 ± 4,21
LD = lactate dehydrogenase: AST :::: aspartate transaminase.
lized abdominal pain. She was 30 weeks pregnant and had not suggestive of the HELLP syndrome, i.e. thrombocytopenia,
received any antenatal care. Fetal movements had ceased 12 haemolysis (micro-angiopathic haemolytic anaemia) and abnor-
hours before admission. The patient stated that she had lost mal liver enzyme values. This is in marked contrast to studies
approximately 40 ml of bright red blood per vaginam. by Weinstein I and Killam ec aP The latter reported 5 cases of
On examination her blood pressure was 165/115 mmHg; pre-eclampsia with classic characteristics of the HELLP syn-
there was proteinuria ++ and oedema localized to the lower drome and commented that the entity is more common than
limbs. The pulse rate was 110/min and she was mildly jaun- realized, that prompt delivery is indicated regardless of gesta-
diced. The respiratory and cardiovascular systems were normal; tional age, and that hypertension does not have to be severe in
on abdominal examination the height of the uterine fundus this syndrome. Pritchard ec al. 4 reported 3 cases in eclamptic
corresponded to 30 weeks' gestation. The uterus felt tense and patients with only 1 survivor. The 16 women presenting with
tender, and the fetal heart was inaudible. Speculum exami- eclampsia in the present study had no haematological features
nation of the lower genital tract revealed no evidence of of the syndrome, which is apparently uncommon in the black
antepartum haemorrhage. An ultrasound examination con- population seen at King Edward VIII Hospital. However, a
firmed intra-uterine death, but there was no evidence of either larger number of women with hypertension of pregnancy need
placenta praevia or abruptio placentae. to be studied for an accurate estimate of the incidence of the
Results of laboratory investigations were: haemoglobin con- syndrome.
centration 19 g/dl, haematocrit 20%, peripheral smear - Platelet consumption is a well-recognized feature of the pre-
schizocytes ++, Burr cells ++, platelet count 51,0 x 10 9/1 - eclampsia/eclampsia syndromes. 5 - 7 Pritchard ec al. 6 reported
22,0 x 109 /1, disseminated intravascular coagulation profile that thrombocytopenia (platelet counts of less than ISO x
normal, Coombs' test (direct and indirect) negative, chest 10 9/1) was present in 28 of 95 women (29%) with eclampsia,
radiograph normal. Liver function test results were as follows: and 16 (17%) had counts of less than I()() x 109 /1. These low
aspartate transaminase (AST) 1386 V/I, lactate dehydrogenase counts rose to normal by the 5th day after delivery and often
(LD) 4334 V/I, bilirubin 56 j..lmol/l. A diagnosis of the then became what he describes as supranormal. We found no
HELLP syndrome was made on the basis of severe pre- statistically significant difference in platelet countS either on
eclampsia and the laboratory findings. day I or day 7 in either group.
Labour was induced and a macerated fetus weighing I 250 g The question whether platelet count falls as normal preg-
was delivered 12 hours later. There was no evidence of a nancy progresses is controversial. Redman ec al. 5 found no
retroplacental clot. Platelet transfusion had been administered changes in platelet count in women who did not develop the
2 hours before delivery, but in the second stage of labour the renal changes of pre-eclampsia. It would seem that the platelet
patient developed acute pulmonary oedema. Her initial response count in pregnant black women needs to be studied in greater
to conventional therapy was unsatisfactory and she required detail, preferably using serial estimations throughout pregnancy
intermittent positive-pressure ventilation in the intensive care as an index of comparison.
unit after delivery. She recovered well and was transferred to The case reported in this study illustrates clearly the prompt
the general posmatal wards on the 2nd day after delivery. disappearance of all abnormal features after delivery. More
She was discharged on day 10 with a normal blood pressure. importantly, the dramatic succession of events in this patient
Pertinent haematological and biochemical parameters were as should make obstetricians aware of the fact that the HELLP
follows: platelet count 351 x 10 9 /1, haematocrir 38%, peripheral syndrome should be suspected in all women with pre-eclampsia,
smear - anisocytes +, AST 16 V/I, LD 298 VII, bilirubin 13 especially if epigastric pain is present. The presence of epigas-
j..lmol/l. tric pain often leads to a non-obstetric diagnosis, and treatment
is withheld or modified. Weinstein 1 found that 90% of his
patients with a diagnosis of HELLP syndrome had epigastric
Identification of these patients is extremely important}
In this study of 26 women with severe hypertensive crises of because the poor fetal and maternal prognosis dictates prompt
pregnancy, 16 of whom had eclampsia, only 1 had features delivery to avoid serious or fatal complications.