HELLP SYNDROME

Subudhi Khetrabasi,Behera Susanta Ku, Subudhi Monalisa Das Sudhansu Ku,Jena Soubhagya Kumar

INTRODUCTION HELLP syndrome affects 0.3% of all pregnancies and 20% of severe preeclampsia & eclampsia, more common in white women than Indian subcontinent. It can occur during antepartum period(2/3rd ) or in postpartum period(1/3rd) most commonly beyond 48 hrs following delivery. The credit of first diagnosis of HELLP syndrome goes to LOUIS WEINSTEIN in 1982.1 PATHOGENESIS It is characterised by microangiopathic hemolytic anemia, consumptive thrombocytopenia, liver dysfunction which also occurs secondary to placental abruption, sepsis or fetal death . The diagnosis is established by three criterias as : i) Hemolysis(H) evedient by abnormal peripherical smear and serum bilirubin >1.2 mg/dl, ii) Elevated Liver (EL) Enzymes shown by rise of serum SGOT/SGPT >72 IU / L and LDH >600 IU / L, iii) Low Platelets(LP) i.e platelet count < 150 103 /mm3 . Common biomarkers to follow the disease progression includes platelet count & serum LDH, HCG, maternal AFP, serum haptoglobin out of which first two carries prognostic significance. Depending upon the platelet count it is graded into three clasess e.g Class 1 TPC <50 000/mm3, Class 2 TPC: 50 000 - 100 000/mm 3 and Class 3 TPC :100 000-150 000/mm3. Complications of this entity includes ARF(5%), ARDS,Pulmonary edema(10%), hemorrhage, placental abruption(10%), liver hematoma with rupture(1.6%) 2 CLINICAL FEATURE Commonly manifested by nausea, vomiting, epigastric pain, and biochemical and hematological changes along with the features of primary pathology like preeclampsia and eclampsia. Two clinical types are presented as 1) Full HELLP syndrome where all the three criteria are found and these are the cases considered for delivery within 48 hours to avoid untoward complications, 2) Partial HELLP Syndrome where some criteria are present, those may be subjected for more conservative management if not accompanied with eclampsia. It is similar

to pre-eclampsia with the features of epigastric pain or right upper quadrant pain, jaundice, microangiopathic anaemia and derranged liver function tests.3 Differential Diagnosis includes thrombotic thrombocytopenic purpura/hemolytic uremic syndrome, disseminated intravascular coagulation or acute fatty liver or Sepsis, Severe hemorrhage Abruptio Placentae, Connective tissue disorders like systemic lupus erythematosus, Primary renal disease- acute glomerulonephritis and diabetes mellitis 4 INVESTIGATIONS Routine investigation like hemoglobin, DC, TLC, TPC, BT/CT, Urine (R & M) and Protein, LFT, RFT, Serum Uric acid, ECG, FBS, ophthalmoscopy, obstetric USG Scan and Special investigations like BPP, CT, CTG, Coagulation Profile, color Doppler, MRI, electrolytes.
MANAGEMENT

It consists of transferring the patient to ICU where safe delivery can be done as delivery is the only cure, bed rest, judicious fluid therapy including crystalloid or albumin-5 to 25%, magnesium sulphate, antihypertensive, correction of electrolyte imbalance. Corticosteroids have established their role in HELLP syndrome and common steroids used are dexamethasone, predinsolone and betamethasone. Usual dose of dexamethasone is 10 mg IV as 12 hrs apart before delivery and 5 mg IV as 12 hrs apart following delivery. Adminstration of corticosteroids improves both maternal outcome (improves thrombocyte count and urine output) and perinatal outcome (improves pulmonary maturity, decreases intraventriculr hemorrhage & necrotising enterocolitis). Steroids are continued till Liver function abnormalities are resolving and TPC > 100,000 per mm3. Absence of improvement of the thrombocytopenia within 72-96 hrs postpartum raises the probability of multi organ failure. 6 Antithrombotics like low dose aspirin & heparin may be given. Indications for termination of pregnancy include a) gestational age of 32-34 weeks, b) bleeding/DIC, c) abruptio placentae, d) eclampsia,e) abnormal FHR pattern. Patients with DIC should be given fresh frozen plasma and packed red blood cells.6 If transabdominal delivery is required, prefer : a ) vertical skin incisin, b) corporeal incision of the uterus, c) spontaneous delivery of placenta to avoid hemorrhage, d) transfuse 6

packs of platelet if < 40000/cmm, e) insert subfascial drain, f) secondary skin closure or leave, g) observe for bleeding from upper abdomen before closure. Regional anesthesia like spinal or epidural preferred for labor or caesarian section and carries decreased maternal morbidity & mortality. Epidural is choice of anesthesia due to provocation of excessive hypotension, superior pain relief, promotes uteroplacental blood flow. It can be extended to provide regional anesthesia for instrumental delivery or caesarian section. General Anesthesia is only indicated in coagulopathy(platelet count < 75000/cmm) / pulmonary edema / impaired consciousness, failed spinal /epidural block. Regular Monitoring of blood pressure, vitals and urine output is done 4 hrly up to which MgSo4 is administered i.e 24 hr of delivery/last Seizure. Antihypertensives are continued till blood pressure is less than 150/100 mm of Hg. Postnatal follow up for 6 weeks is done. Maternal Mortality and perinatal mortality can be up to 50%and 25% respectively in untreated HELLP Syndrome where as rate of recurrence in subsequent pregnancy ranges from 2% to 19%. So rational diagnosis and management in experinced hand can save the life of mother and baby.7 BIBLIOGRAPHY 1) Fernado Arias. Hypertensive Disorder in Pregnancy .Practical Guide to High Risk Pregnancy..
3rd Edn; 2008; Ch-16.P-427-28.

2) Kaur A P. HELLP Syndrome associated with Moderate to Severe PIH/ Eclampsia. J Obste
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3) Fischer T. Vascular Reactivity in patients with preeclampsia and HELLP syndrome. Am J Obste
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4) Sibai B M. Pregnancies Complicated by HELLP syndrome: Subsequent outcome and Long term
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5) Martin J N. Maternal Benefit of high dose intravenous Corticosteroid therapy for HELLP
syndrome. Am J Obst. Gynecol.2005; 189:830.

6) Hauth J C. Low dose aspirin therapy to prevent Preeclampsia. Am J Obst. Gynecol ; 2005:
168;1083-1993

7) Ramadan M K. Maternal Morbidity and Mortality in 442 pregnancies with HELLP. Am J Obste
Gynecol; 2003b. 169:1000.