Thrombotic Thrombocytopenic Purpura Masquerading as Hemolysis, Elevated Liver Enzymes, Low Platelets (HELLP) Syndrome in Late Pregnancy

Microangiopathic antiphospholipid-associated syndromes (MAPSs) are reported as encompassing several conditions mainly affecting the microvasculature of selected organs: the liver in HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet); kidney, brain, and skin in TTP (thrombotic thrombocytopenic purpura). It is predominant in patients with catastrophic antiphospholipid syndrome (APS). A recent report suggests that APS is not only a thrombotic disease but also associated with microangiopathic features, and it can explain the greater prevalence of HELLP syndrome in these patients. We here report a case of MAPS during pregnancy associated with systemic lupus erythematosus (SLE) in early second trimester.

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Thrombotic thrombocytopenic purpura and HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome: Differential diagnostic problems

American Journal of Obstetrics and Gynecology ◽

10.1016/s0002-9378(96)70179-5 ◽

1996 ◽

Vol 175 (2) ◽

pp. 506-507 ◽

Cited By ~ 2

Author(s):

C. Kaiser ◽

W. Distler

Keyword(s):

Thrombotic Thrombocytopenic Purpura ◽

Liver Enzymes ◽

Thrombocytopenic Purpura ◽

Diagnostic Problems ◽

Elevated Liver Enzymes

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

Journal of the Intensive Care Society ◽

10.1177/17511437211025410 ◽

2021 ◽

pp. 175114372110254

Author(s):

Evangelia Poimenidi ◽

Yavor Metodiev ◽

Natasha Nicole Archer ◽

Richard Jackson ◽

Mansoor Nawaz Bangash ◽

...

Keyword(s):

Acute Kidney Injury ◽

Critical Care ◽

Liver Failure ◽

Hellp Syndrome ◽

Seizure Control ◽

Liver Enzymes ◽

Kidney Injury ◽

Definitive Treatment ◽

Multisystem Disease ◽

Elevated Liver Enzymes

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.

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