What is HELLP syndrome ??

How many members have come across in last 10 years ? May kindly post your experiences about the methodology of diagnois & outcome please .What is HELLP Syndrome?? Let us refresh our knowledge on HELLP

Q. How do we define HELLP ?? Ans:  From text Books we know that HELLP syndrome is a variant of preeclampsia  and defined by the  following criteria : Point 1:--Hemolysis  identified  by burr cells , helmet cells,   and schistocytes on an abnormal  peripheral  smear   an elevated serum  Bilirubin  or LDH  level  or a low serum haptoglobin. Point 2:-- Thrombocytopenia with platelets  <  100,000  /uL  is the most consistent finding in HELLP   syndrome.   INR elevated ( 1.9)
 Point 3: Elevated liver  function tests(enzymes in particular) However bilirubin also rises and it is LDH which is high > 600 IU//Liters and also concurrent elevation of > 70 IU/Lit (.   greater than two times  the upper  limit of normal).   , We should note that the hypertension  may be absent/   mild  or severe  and surprisingly Proteinuria may be absent  as well . But, Deranged renal function ( creatinine 1.6) is not uncommon
Therefore Lab features will be  Hemolysis  elevated liver  enzymes and low platelet syndrome  is the most common  pathologic cause of maternal thrombocytopenia . It occurs in approximately  105  to 205  of women who have  severe  preeclampsia and is often an early  finding in preeclampsia. Platelets  usually  nadir at 24 to 48  hours  after delivery  but typically do not drop below  20,000 /uL .

Etiology of HELLP  ? Why such an deadly event happens? Ans:-Somehow or other there is hepatic endothelial cell damage. Followed by platelate activation and platelate activation à aggregation of platelatesàSatiation of platelate at capillaries in diff parts of bodyà Cellular death./

Q. 2: Clinical Features ?? How does such syndrome present??

HELLP syndrome often  presents with nonspecific  complaints such as  1) malaise2)   abdominal  pain more epigastric region 3 vomiting  4) shortness of breath or bleeding

Q  3:  Differential  diagnosis ?  What diseases and syndrome may mimic HELLP ?? The differential  diagnosis for HELLP  syndrome includes

Acute  fatty liver  of pregnancy

Thrombotic   thrombocytopenic purpura

Hemolytic uremic syndrome

Immune thrombocytopenic  purpura

Systemic lupus   erythematosus flare

Antiphospholipid  antibody  syndrome 

Cholecystitis

Fulminant hepatitis

Acute   pancreatitis

Disseminated herpes zoster

Q.  4 How to treat??  Ans: Management is the same as for severe  preeclampsia . 1) Control of BP if HTN associated 2) Neuroprotection 3) Prophylaxis for convulsion 4) Correction of coagulation disorders like platelet transfusion may be required immediately   prior  to delivery  depending  on severity  of thrombocytopenia. 5) Short term  expectant management in order to allow  for administration of beta methadone for fetal  lung maturity  may be possible  in a very  selected  group of patients  with HELLP  prior to 34 weeks however   there are  no data  suggesting improved  perinatal  outcomes  with this approach.

Q.6 HELLP  syndrome with severe thrombocytopenia-How to manage ??

In women who remain severely thrombocytopenic after delivery . Plasma exchange and / or  corticosteroids may be considered. In addition to improving  maternal  outcomes. When given   in the antepartum period  transient  improvements  are see in maternal  platelet counts . small  controlled  trials have not shown decreased morbidity when steroids are continued postpartum .

Take home message : HELLP can present with varying symptoms & signs and  as mentioned  hepatic endothelial     damage and platelate  activation and aggregation of platelates may quickly ensue within short span of time

. HELLP lab markers are often variable and  we can be, , that can masquerade as anything else ! We usually think of usual and known problems, which is the right way of thinking, yet, rarely, such surprises hit us. Obstetrics is tough, since with medical and surgical diseases occurring alongside, creating symptoms and signs that can be seen as normal for pregnancy, we can miss things, sometimes with serious fallouts. Pregnancy complicates several such diseases, worsening them too, with high mortality/morbidity. Luck saves that day many a time ! I am thankful for this learning