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[edit | edit source]

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[edit | edit source]

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

Laboratory[edit | edit source]

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[edit | edit source]

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[edit | edit source]

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

Complications[edit | edit source]

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

Treatment[edit | edit source]

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 ).