HELLP syndrome

HELLP syndrome is a very serious complication of pregnancy with significant mortality and morbidity (up to 40%). The name originated from the English words Hemolysis, Elevated Liver enzymes, Low Platelets. It is described either as a separate disease or as a complication of preeclampsia. It is a state of severe preeclampsia complicated by hemolysis, thrombocytopenia , abnormal peripheral blood smear, and abnormal liver function tests.

Incidence
In 2-12% of preeclampsia, most often in multiparous couples around the age of 25, most often in the 36th week, in 70% before delivery, in 30% after delivery.

Pathogenesis
Unclear, probably generalized vasospasm with subsequent MODS (mainly liver, kidney ) and activation of coagulation. The result is microangiopathic thrombocytopenia.

Laboratory

 * Hemolysis – pathological smear of blood – there are schistocytes and burr cells (triangular erythrocytes ) – probably formed by flow through the affected vessel, rise of bilirubin above 20 μmol/l, LD (5×), free iron in the serum.
 * Elevation of liver tests – mainly AST (2–3×).
 * Thrombocytopenia - drop below 100, consumption and destruction.

Clinical picture
Pain in the epigastrium and in the right hypochondrium, nausea and vomiting dominate. Prodromes – non-specific, flu-like (malaise, fatigue). The advanced stage is manifested by bleeding ( hematuria, bleeding into the GIT ). Weight gain with swelling, hypertension with proteinuria , sometimes jaundice. A fully developed HELLP mimics DIC.

Differential diagnosis
Acute hepatic steatosis, ITP, HUS , hyperemesis gravidarum, cholecystopathy, viral hepatitis , gastroenteritis , appendicitis , pyelonephritis , renal colic , SLE.

Complications
DIC (up to 40%), placental abruption, acute respiratory insufficiency, ARDS , ascites , rupture of subcapsular hematoma of the liver, eclampsia , IUGR.

Treatment
Termination of pregnancy is the only causal treatment. Conservative therapy is for the postpartum period. Expansion of plasma volume, adjustment of coagulation ( antithrombin III, plasma , erymasa, platelets), corticoids (immunosuppression), anticonvulsant prophylaxis (MgSO 4 , valium), antihypertensives , ATB , diet, hepatoprotectants , plasmapheresis ( dialysis ).

Related Articles

 * Hypertension in pregnancy
 * Preeclampsia
 * Eclampsia
 * Hypertension
 * Antihypertensives
 * Birth