Haemolysis, elevated liver enzymes and low platelets: Diagnosis and management in critical care

Abstract

A thirty-year-old pregnant woman was admitted to hospital with headache and gastrointestinal discomfort. She developed peripheral oedema and had an emergency caesarean section following an episode of tonic-clonic seizures. Her delivery was further complicated by postpartum haemorrhage and she was admitted to the Intensive Care Unit (ICU) for further resuscitation and seizure control which required infusions of magnesium and multiple anticonvulsants. Despite haemodynamic optimisation she developed an acute kidney injury with evidence of liver damage, thrombocytopenia and haemolysis. Haemolysis, Elevated Liver enzymes and Low Platelets (HELLP) syndrome, a multisystem disease of advanced pregnancy which overlaps with pre-eclampsia, was diagnosed. HELLP syndrome is associated with a range of complications which may require critical care support, including placental abruption and foetal loss, acute kidney injury, microangiopathic haemolytic anaemia, acute liver failure and liver capsule rupture. Definitive treatment of HELLP is delivery of the fetus and in its most severe forms requires admission to the ICU for multiorgan support. Therapeutic strategies in ICU are mainly supportive and include blood pressure control, meticulous fluid balance and possibly escalation to renal replacement therapy, mechanical ventilation, neuroprotection, seizure control, and management of liver failure-related complications. Multidisciplinary input is essential for optimal treatment.

Ruptured Subcapsular Liver Hematoma: A Rare Complication of HELLP Syndrome

“…It is important to consider alternative diagnoses such as microangiopathic hemolytic anemia, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura, which can be distinguished from HELLP syndrome by the significant increase in liver enzymes and the absence of proteinuria. [6][7][8] Although the exact etiology of HELLP syndrome is unknown, many studies suggest an immunological role in its pathogenesis. Endothelial injury, vascular spasm, and platelet aggregation are believed to play important roles in the inflammatory reaction that leads to hepatic sinusoidal obstruction, ischemia, and necrosis.…”

“…Hepatic rupture can occur when intrahepatic pressure increases beyond the capacity of Glisson's capsule. [8][9][10] To successfully manage ruptured subcapsular liver hematoma, a multidisciplinary team consisting of medical, surgical, obstetrical, and radiology specialists is necessary. The initial step should be to provide good resuscitation with blood transfusions and intensive care management.…”

HELLP Syndrome with Unusual Features in a Rare Setting

“…HELLP syndrome is the acronym used to describe a constellation of Hemolysis, Elevated Liver enzymes, and Low Platelet syndrome [1][2][3]. The syndrome is an adverse complication at the severe end of hypertensive disorders of pregnancy [2,3].…”

“…HELLP syndrome is the acronym used to describe a constellation of Hemolysis, Elevated Liver enzymes, and Low Platelet syndrome [1][2][3]. The syndrome is an adverse complication at the severe end of hypertensive disorders of pregnancy [2,3]. It occurs in 0.5-0.9% of all pregnancies but at a higher rate when it is associated with severe preeclampsia (10-20%) [4].…”

“…The exact pathophysiology of HELLP syndrome remains ill-defined, but evidence from several studies supports the involvement of several theories [7]. Risk factors include multiparty, European descent/Caucasian background, advanced maternal age older than 34 years, and poor pregnancy outcomes, which are all characteristics at variance with the index case [2,11].…”