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Folate
deficiency is common in pregnancy. HELLP syndrome. H = haemolysis. EL =
Elevated liver enzymes.
LP = low platelets is a pregnancy complication which affects 10 - 20%
cases of severe pre-eclampsia. Routine supplements of folic acid in high
doses is recommended. Careful scrutiny of clinical laboratory findings
may help to discriminate the HELLP syndrome from its mimics avoiding pre-term
delivery. |
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Introduction
Folate defi ciency is common in pregnancy, progression to megaloblastosis
is not.
Haemolytic anaemia, thrombocytopenia and coagulopathy due to folate deficiency
may mimic the syndrome of haemolysis, elevated liver enzymes and low platelets
(HELLP). The HELLP syndrome1 H = Haemolysis EL = elevated liver enzymes.
LP = low platelets is a pregnancy complication which affects 10-20% cases
of severe pre-eclampsia.2
Symptoms and signs are non-specific until overt folate lack with megaloblastic
anaemia occurs. In western countries the incidence of megaloblastic anaemia
in pregnancy is 0.3%.3 whereas the incidence in India is as high as 10-20%.4
We
present here three cases of folic acid deficiency showing similarity with
HELLP syndrome from the gynaec and obstetric OPD of Shri Mumbadevi Homeopathic
Hospital. |
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CASE
1
A 28 year old, 2nd gradiva was admitted at 30 weeks period of gestation
with history of upper abdominal discomfort,
vomiting, bleeding per vagina. O/E pallor ++ tachycardia, BP 140/100 mm
Hg in supine position.
Abdominal Examination - Uterus 30-32 weeks, single foetus in cephalic
presentation. FHS were feable fast. Bleeding per vagina.
Investigation - Haemoglobin 4.8 gm%, Total leucocyte count 4,500/cumm.
BT 8 mins CT 10 mins, Prothrombin time normal, Partial thromboplastin
time - normal. Platelets 80,000/cumm. Urinary Albumin : Present ++. Folic
acid levels - 5 mg/ml
Looking at the spectrum of findings, headache, vomiting, raised BP, pain
in abdomen, albuminuria, bleeding and
thrombocytopenia, a provisional diagnosis of HELLP syndrome was made. |
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CASE
2
A 35 year old, multigradiva was admitted at 28 weeks period of gestation
with history of vomiting, epistaxis, bleeding per
vagina and haematuria O/E pallor ++, petechial haemorrhage on skin and
tongue. The size of the uterus corresponds to the period of gestation
and FHS were fair.
Investigation - Haemoglobin 4.8 gms% PVC 15 MCV 105, platelet count 70,000/cumm.
Peripheral smear showed dimorphic anaemia with plated reduced on smear,
hypersegmented neutrophils. Folic acid levels - 4.8 mg/ml
BT - normal, CT prolonged, Prothrombin time - prolonged, LDH increased.
Serum Bilirubin mildly rised (2 mg%)
considering the striking similarity in clinical picture as the previous
case and similar laboratory investigation HELLP
syndrome was considered as the provisional diagnosis. |
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CASE
3
25 years multigravida was admitted at 36 weeks for bleeding per vagina,
pain in upper abdomen, nausea, vomiting with
breathlessness. O/E Pallor ++ Haemoglobin 4.0 gm% MCV 115 PCV 16, platelets
decreased 40,000/cumm. Peripheral smear macrocytosis with hypersegmented
neutrophils. LDH increased. Bilirubin 2.5 mg%. Reticulocytosis 5%. Serum
folate level were decreased. Provisional diagnosis of macrocytic anaemia
was made. Necessary blood transfusion was given. An LSCS was performed,
Healthy Baby of 2.5 kg was delivered, patient had uneventful recovery
and was discharged after 7 days. The patient was readmitted on the 8th
day of LSCS in a toxic state having WBC count of 36500/cumm. Hb 3.5 gm%
RBC 1.0 million/cumm PCV 9 MCV 118 Peripheral smear showing, schistocytes
Fragments of RBC, macrocytosis with reduced platelets on smear. Blood
culture - showed no organism. Urine - Albumin ++, Urobilinogen strongly
positive. Creatinine, Blood Urea nitrogen - normal.
Electrolytes Na - 140 mEq/L, K - 35 mEq/L CL - 100 mEq/L. S. Bilirubin
3.0 mg% folate level 4 mg/ml.
On this basis a provisional diagnosis of autoimmune haemolytic anaemia
was made by excluding lymphoma,
leukaemia since there was no organomegaly and no abnormal cells seen on
smear. In view of this we thought of SLE. Which was confirmed by the presence
of LE cells. The patient was managed by Blood transfusion and folic acid
supplements. The patient was asked to follow up in OPD for further investigation.
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DISCUSSION
In 1982 Weinstein5 coined the term HELLP syndrome to describe a special
group of preeclamptic women, who had evidence of haemolysis, elevated
liver enzymes, low platelets. The question of whether the HELLP syndrome
exists as a distinct entity or is a part of a spectrum of pregnancy complication,
has long been a source of speculation and debate among obstetricians and
internists.
A review of the literature indicates that the syndrome occurs in post-partum
from 20-30% of cases, maternal mortality from 1 to 4% and perinatal mortality
from 5 to 40%. Haemolytic anaemia, thrombocytopenia and coagulopathy due
to folate deficiency may mimic the syndrome of haemolysis, elevated liver
enzymes and low platelets. The serious complication of folate deficiency
make a strong case for supplementation in pregnancy. Careful scrutiny
of clinical and laboratory findings may help to discriminate the HELLP
syndrome from its mimics avoiding preterm delivery.
Clinical features of severe folate deficiency vary from fulminating6-7
cases of severe anaemia to jaundice, upper bdominal pain and bleeding
from various body sites. One of the earliest laboratory markers of folic
acid deficiency is low serum folate concentration at 3rd week of folate
deprivation. Increased excretion of forminioglutamic acid (FIGLU) appear
later followed by fiorid picture of megaloblastosis in the bone marrow
at over 19-20 weeks of folate deprivation. As megalobalstosis processes
there will be pancytopenia with elevated serum bilirubin, LDH, due to
intramedullary
haemolysis1 the diagnosis in pregnancy is masked because of co-existent
iron deficiency and therefore
bone marrow examination is essential. In view of potential complication
of folic acid deficiency in pregnancy routine supplementation is recommended
in higher dose. The high dose which is administered 2 to 3 months before
conception has been proven to prevent occurrence of neural tube defect
in foetus.8
The patients presented with severe anaemia, jaundice and pain in abdomen
with haemorrhagic manifestation, further more they had dimorphic anaemia,
thrombocytopenia, increased bilirubin and mild deranged coagulopathy,
all these features
favoured the diagnosis of HELLP syndrome. However the two cases improved
by supplementation of folic acid which resulted in favourable maternal
and foetal outcome. The third case was also mimicking HELLP syndrome,
but
was readmitted in toxic state and re-evaluation of case revealed SLE provisionally
and the underlying cause of anaemia, which was proved by peripheral smear
showing LE cells, but however such patients have to be screened in detail,
not to miss autoimmune cause of haemolysis (Cardiolipin Antibodies).9
The most reliable laboratory tests for the diagnosis
of HELLP10 syndrome are complete blood count with peripheral smear examination,
LDH, SGPT, Urinalysis, supportive test include serum hepatoglobin, D-dimer
fragment levels, isoenzyme of LDH, prothrombin time, bilirubin, platelets,
LDH and platelets are the two best tests to monitor the course of the
disease. The intensity of HELLP syndrome peaks 24 hours after delivery.
Extended atypical HELLP has been successfully treated with plasma exchange.
Therefore every pregnant woman should receive dietary advice early in
pregnancy11 so anaemia could be avoided. The clinical laboratory
professional plays an important role in the diagnosis, follow up and treatment
of patient with folate deficiency mimicking HELLP syndrome.10 Hence one
should be cautious in labeling HELLP syndrome which has poor prognosis,
with termination of pregnancy being the mainstay in the management. The
present study of cases shows how simple
condition like folate deficiency may lead to life threatening complication
and ascertain the importance of routine folate supplementation in early
pregnancy. |
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ACKNOWLEDGEMENT
We are thankful to the Dean Dr. SK Goel for permitting us to publish the
data. We express our sincere gratitude to the staff of the Pathology Dept.
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REFERENCES
1. Haran K Bjorge, L Guttu K. HELLP syndrome. Pidsskr Nor Laegeforen 2000;10,
120, 12 : 1433-6.
2. Wein P, et al. Severe folate deficiency masquerading as the syndrome
of hemolysis, elevated liver enzymes and low
platelets. Am J Obst and Gynac 1997; 90 : 655-7.
3. Louis ST. CV Mosby Medical disorders during pregnancy 2nd ed. 1995;
228-69.
4. Orient longman. Post Graduate obstetrics and gynecology 4th ed. Hyderabad
1989; 60-8.
5. D’Anna R. The HELLP syndrome Nos on its pathogenesis and treatment.
Minerva Ginecal 1996; 48 (4) : 47-54.
6. Raju GR. Folic acid deficiency in megaloblastic anemia obstetrics and
fetal medicine 1st ed New Delhi, S Chand
and Company 1993; 159-63.
7. Rush B, et al. Clinical hematology in medical practice 5th ed Delhi
Oxford University press 1990; 62-101.
8. Scott M, Weir DG. Role of folate in pregnancy pervention. Better than
cure. Recent advances in obstetrics and
gynecology No 20 London 9. Churchill Livingston 1998; 1-20.
9. Harris EN, Spinnato JA. Should anti-cardiolipin tests be performed
in otherwise healthy pregnant womenfi Am J
Obstet Gynecology 1991; 165 (4PT 1) : 1154-5.
10. Jones SL. HELLP A Cry for laboratory assistance, a comprehensive review
of HELLP syndrome highlighting
the role of the Laboratory. Hematopathol Mol Hematol 1998; 11 (3-4) :
147-71.
11. Ehingsen TJ, Sommer S. Macrocytic anemia in the last trimester of
pregnancy due to dietary insufficiency-initially
interpreted as the HELLP syndrome. Ugeokr Laeger 1967-68 : 28 : 156. |
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PILL
COULD REDUCE CADIOVASCULAR DISEASE CONSIDERABLY
Wald and colelagues present the concept of combining six active
components in one pill (the Polypill) taken every day
from age 55, or sooner if people have a diagnosis of cardiovascular
disease or diabetes. They argue that the combination of a statin,
a thiazide, a b blocker, an angiotensin converting enzyme inhibitor,
folic acid, and aspirin simulataneously reduces four key cardiovascular
risk factors; low density lipoprotein cholesterl, blood pressure,
serum homocysteine, and platelet function.
In an acompanying editorial, Rodgers says that the Polypill may
have enormous potential for preventing cardiovascular
disease, including in developing countries.
BMJ, 2003; 326 : 1419. |
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